283

Tetraspanin 15 Depletion impairs Extracellular Vesicle Docking with Target Neurons
Stajano D, Lombino FL, Schweizer M, Glatzel M, Saftig P, Gromova KV, Kneussel M (2023)
J. Extracell. Biol., in press.

282

Lysosome-targeted multi-functional lipid probes reveal sterol transporters NPC1 and LIMP-2 as sphingosine transporters.
Altuzar J, Notbohm J, Stein F, Haberkant P, Hempelmann P, Heybrock S, Worsch J, Saftig P, Höglinger D (2022)
Proc. Natl. Acad. Sci U.S.A. 2023 Mar 14;120(11):e2213886120.

281

Mechanisms of amyloid-β34 generation indicate a pivotal role for BACE1 in amyloid homeostasis.
Ulku I, Liebsch F, Akerman SC, Schulz JF, Kulic L, Hock C, Pietrzik C, di Spiezio A, Thinakaran G, Saftig P, Multhaup G. (2023)
2023 Feb 7;13(1):2216.

280

Consecutive functions of small GTPases guide HOPS-mediated tethering of late endosomes and lysosomes.
Schleinitz A, Pöttgen LA, Keren-Kaplan T, Pu J, Saftig P, Bonifacino JS, Haas A, Jeschke A.
Cell Rep. 2023 Jan 31;42(1):111969.

279

ADAM10-dependent Notch signaling establishes dental cell boundaries required for enamel formation.
Mitsiadis TA, Jimenez-Rojo L, Weber S, Saftig P, Pagella P. (2022)
iScience, 2022 Sep 16;25(10):105154.

278

LAMP2 regulates autophagy in the thymic epithelium and thymic stroma-dependent CD4 T cell development.
Rodrigues PM, Sousa LG, Perrod C, Maceiras AR, Ferreirinha P, Pombinho R, Romera-Cárdenas G, Gomez-Lazaro M, Senkara M, Pistolic J, Cabanes D, Klein L, Saftig P, Alves NL.
Autophagy. 2023 Feb;19(2):426-439.

277

Phagosomal signalling of the C-type lectin receptor Dectin-1 is terminated by intramembrane proteolysis.
Mentrup T, Stumpff-Niggemann Y, Leinung N, Schlosser C, Schubert K, Wehner R, Tunger A, Schatz V, Neubert P, Gradtke AC, Wolf J, Rose-John S, Saftig P, Dalpke A, Jantsch J, Schmitz M, Fluhrer R, Jacobsen ID, Schröder B.
Nature Comm. 2022 Apr 6;13(1):1880

276

Lysosomal cathepsin D mediates endogenous mucin glycodomain catabolism in mammals.
Pedram, K, Laqtom, NN, Shon, DJ, Di Spiezio A, Riley NM, Saftig P, Abu-Remaileh M, Bertozzi CR.
Proc. Natl. Acad. Sci. 2022 Sep 27;119(39):e2117105119.

275

Intravitreal gene therapy restores the autophagy-lysosomal pathway and attenuates retinal degeneration in cathepsin D-deficient mice.
Liu J, Bassal M, Schlichting S, Braren I, Di Spiezio A, Saftig P, Bartsch U.
Neurobiol Dis. 2022 Mar;164:105628

274

Lung emphysema and impaired macrophage elastase1 clearance in mucolipin 3 deficient mice.
Spix B, Butz ES, Chen CC, Scotto Rosato A, Tang R, Jeridi A, Kudrina V, Plesch E, Wartenberg P, Arlt E, Briukhovetska D, Ansari M, Günsel GG, Conlon TM, Wyatt A, Wetzel S, Teupser D, Holdt LM, Ectors F, Boekhoff I, Boehm U, García-Añoveros J, Saftig P, Giera M, Kobold S, Schiller HB, Zierler S, Gudermann T, Wahl-Schott C, Bracher F, Yildirim A, Biel M, Grimm, C.
Nat Commun. 2022 Jan 14;13(1):318

273

Recombinant pro-Cathepsin D enhances alpha-Synuclein degradation in alpha-Synucleinopathy models.
Huarcaya SP, Drobny A, Marques ARA Di Spiezio A, Dobert JP, Balta D, Werner C, Rizo T, Gallwitz L, Bub S, Stojkovska I, Belur NR, Fogh J, Mazzulli JR, Xiang W, Fulzele A, Dejung M, Sauer M, Winner B, Rose-John S, Arnold P, Saftig P, Zunke F.
Autophagy, 2022 May;18(5):1127-1151.

272

Inhibition of ADAM17 impairs endothelial cell necroptosis and blocks metastasis.
Bolik J, Krause F, Stevanovic M, Gandraß M, Thomsen I, Schacht S, Rieser E, Müller M, Schumacher N, Fritsch J, Wichert R, Galun E, Bergmann J, Röder C, Schafmayer C, Egberts JH, Becker-Pauly C, Saftig P, Lucius R, Schneider-Brachert W, Barikbin R, Adam D, Voss M, Hitzl W, Krüger A, Strilic B, Sagi I, Walczak H, Rose-John S, Schmidt-Arras D.
J. Exp. Med. 2022 Jan 3;219(1):e20201039

271

S-Palmitoylation determines TMEM55B-dependent positioning of lysosomes.
Rudnik S, Heybrock S, Saftig P, Damme M.
J Cell Sci. 2022 Mar 1;135(5)

270

Post-translational modification by ADAM10 shape myeloid antigen presenting cell homeostasis in the splenic marginal zone.
Diener N, Fontaine J-F, Klein M, Hieronymus T, Wanke F, Kurschus F, Ludwig A, Ware C, Saftig P, Bopp T, Clausen BE, Backer RA.
Proc Natl Acad Sci U S A. 2021 Sep 21;118(38):e2111234118

269

LAMP3 deficiency affects surfactant homeostasis in mice.
Lunding LP, Krause D, Stichtenoth G, Stamme C, Lauterbach N, Hegermann J, Ochs M, Schuster B, Sedlacek R, Saftig P, Schwudke D, Wegmann M, Damme M.
Plos Genetics 2021 Jun 23;17(6):e1009619

268

GPR37 is processed in the N-terminal ectodomain by ADAM10 and furin.
Mattila SO, Tuhkanen HE, Lackman JJ, Konzacka A, Morató X, Argerich J, Saftig P, Ciruela F, Petäjä-Repo UE.
FASEB J. 2021 Jun;35(6):e21654

267

Rapid and progressive loss of multiple retinal cell types in cathepsin D-deficient mice, an animal model of CLN10 disease.
Bassal M, Liu J, Jankowiak W, Saftig P, Bartsch U.
Cells. 2021 Mar 21;10(3):696.

266

ADAM10-mediated ectodomain shedding is an essential driver of podocyte damage.
Sachs M, Wetzel S, Reichelt J, Sachs W, Schebsdat L, Zielinski S, Seipold L, Heintz L, Müller SA, Kretz O, Lindenmeyer M, Wiech T, Huber TB, Lüllmann-Rauch RL, Lichtenthaler S., Saftig P*, Meyer-Schwesinger C.*
J. Am. Soc. Nephrol. 2021 Jun 1;32(6):1389-1408 (* equal senior authorship and correspondence)

265

ADAM10 hyperactivation acts on Piccolo to deplete synaptic vesicle stores in Huntington's disease.
Cozzolino F, Vezzoli E, Cheroni C, Besusso D, Conforti P, Valenza M, Iacobucci I, Monaco V, Birolini G, Bombaci M, Falqui A, Saftig P, Rossi R, Monti M, Cattaneo E, Zuccato C.
Hum. Mol. Gen. 2021 Jun 17;30(13):1175-1187

264

LAMP-2 Is Involved in Surface Expression of RANKL of Osteoblasts In Vitro.
Jansen IDC, Tigchelaar-Gutter W, Hogervorst JMA, de Vries TJ, Saftig P, Everts V.
Int J Mol Sci. 2020 Aug 25;21(17):6110

263

Cathepsin D regulates cerebral Aβ42/40 ratios via differential degradation of Aβ42 and Aβ40.
Suire CN, Abdul-Hay SO, Sahara T, Kang D, Brizuela MK, Saftig P, Dickson DW, Rosenberry TL, Leissring MA.
Alzheimers Res Ther. 2020 Jul 6;12(1):80

263

The tetraspanin Tspan15 is an essential subunit of an ADAM10 scissor complex.
Koo CZ, Harrison N, Noy PJ, Szyroka J, Matthews AL, Hsia HE, Müller SA, Tüshaus J, Goulding J, Willis K, Apicella C, Cragoe B, Davis E, Keles M, Malinova A, McFarlane TA, Morrison PR, Nguyen HTH, Sykes MC, Ahmed H, Di Maio A, Seipold L, Saftig P, Cull E, Pliotas C, Rubinstein E, Poulter NS, Briddon SJ, Holliday ND, Lichtenthaler SF, Tomlinson MG.
J Biol Chem. 2020 Sep 4;295(36):12822-12839

262

Enzyme replacement therapy with recombinant pro-CTSD (cathepsin D) corrects defective proteolysis and autophagy in neuronal ceroid lipofuscinosis.
Marques ARA, Di Spiezio A, Thießen N, Schmidt L, Grötzinger J, Lüllmann-Rauch R, Damme M, Storck SE, Pietrzik CU, Fogh J, Bär J, Mikhaylova M, Glatzel M, Bassal M, Bartsch U, Saftig P.
Autophagy. 2020 May;16(5):811-825

261

The FTLD Risk Factor TMEM106B Regulates the Transport of Lysosomes at the Axon Initial Segment of Motoneurons.
Lüningschrör P, Werner G, Stroobants S, Kakuta S, Dombert B, Sinske D, Wanner R, Lüllmann-Rauch R, Wefers B, Wurst W, D'Hooge R, Uchiyama Y, Sendtner M, Haass C, Saftig P, Knöll B, Capell A, Damme M.
Cell Rep. 2020 Mar 10;30(10):3506-3519

260

Systemic AAV9.LAMP2B injection reverses metabolic and physiologic multiorgan dysfunction in a murine model of Danon disease.
Manso AM, Hashem SI, Nelson BC, Gault E, Soto-Hermida A, Villarruel E, Brambatti M, Bogomolovas J, Bushway PJ, Chen C, Battiprolu P, Keravala A, Schwartz JD, Shah G, Gu Y, Dalton ND, Hammond K, Peterson K, Saftig P, Adler ED.
Sci Transl Med. 2020 Mar 18;12(535):eaax1744.

259

Genetic LAMP2 deficiency accelerates the age-associated formation of basal laminar deposits in the retina.
Notomi S, Ishihara K, Efstathiou NE, Lee JJ, Hisatomi T, Tachibana T, Konstantinou EK, Ueta T, Murakami Y, Maidana DE, Ikeda Y, Kume S, Terasaki H, Sonoda S, Blanz J, Young L, Sakamoto T, Sonoda KH, Saftig P, Ishibashi T, Miller JW, Kroemer G, Vavvas DG.
Proc Natl Acad Sci U S A. 2019 Nov 19;116(47):23724-23734

258

Meprin β induces activities of A disintegrin and metalloproteinases 9, 10, and 17 by specific prodomain cleavage.
Wichert R, Scharfenberg F, Colmorgen C, Koudelka T, Schwarz J, Wetzel S, Potempa B, Potempa J, Bartsch JW, Sagi I, Tholey A, Saftig P, Rose-John S, Becker-Pauly C.
FASEB J. 2019 Nov;33(11):11925-11940

257

The lysosomal transporter MFSD1 is essential for liver homeostasis and critically depends on its accessory subunit GLMP.
Massa López D, Thelen M, Stahl F, Thiel C, Linhorst A, Sylvester M, Hermanns-Borgmeyer I, Lüllmann-Rauch R, Eskild W, Saftig P, Damme M.
Elife. 2019 Oct 29;8

256

Lysosomal integral membrane protein-2 (LIMP-2/SCARB2) is involved in lysosomal cholesterol export.
Heybrock S, Kanerva K, Meng Y, Ing C, Liang A, Xiong ZJ, Weng X, Ah Kim Y, Collins R, Trimble W, Pomès R, Privé GG, Annaert W, Schwake M, Heeren J, Lüllmann-Rauch R, Grinstein S*, Ikonen E*, Saftig P*, Neculai D*.
Nat Commun. 2019 Aug 6;10(1):3521
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255

Presynaptic Endosomal Cathepsin D Regulates the Biogenesis of GABAergic Synaptic Vesicles.
Li X, Qin L, Li Y, Yu H, Zhang Z, Tao C, Liu Y, Xue Y, Zhang X, Xu Z, Wang Y, Lou H, Tan Z, Saftig P, Chen Z, Xu T, Bi G, Duan S, Gao Z.
Cell Rep. 2019 Jul 23;28(4):1015-1028.e5

254

Lysosomal degradation of newly formed insulin granules contributes to β cell failure in diabetes.
Pasquier A, Vivot K, Erbs E, Spiegelhalter C, Zhang Z, Aubert V, Liu Z, Senkara M, Maillard E, Pinget M, Kerr-Con­te J, Pattou F, Marciniak G, Ganzhorn A, Ronchi P, Schieber NL, Schwab Y, Saftig P, Goginashvili A, Ricci R.
Nat Commun. 2019 Jul 25;10(1):3312

253

Aβ34 is a BACE1-derived degradation intermediate associated with amyloid clearance and Alzheimer's disease progression.
Liebsch F, Kulic L, Teunissen C, Shobo A, Ulku I, Engelschalt V, Hancock MA, van der Flier WM, Kunach P, Rosa-Neto P, Scheltens P, Poirier J, Saftig P, Bateman RJ, Breitner J, Hock C, Multhaup G.
Nat Commun. 2019 May 20;10(1):2240

252

Inhibiting pathologically active ADAM10 rescues synaptic and cognitive decline in Huntington's disease.
Vezzoli E, Caron I, Talpo F, Besusso D, Conforti P, Battaglia E, Sogne E, Falqui A, Petricca L, Verani M, Martufi P, Caricasole A, Bresciani A, Cecchetti O, Rivetti di Val Cervo P, Sancini G, Riess O, Nguyen H, Seipold L, Saftig P, Biella G, Cattaneo E, Zuccato C.
J Clin Invest. 2019 May 6;129(6):2390-2403

251

Ubiquitin C-terminal hydrolase L1 (UCH-L1) loss causes neurodegeneration by altering protein turnover in the first postnatal weeks.
Reinicke AT, Laban K, Sachs M, Kraus V, Walden M, Damme M, Sachs W, Reichelt J, Schweizer M, Janiesch PC, Duncan KE, Saftig P, Rinschen MM, Morellini F, Meyer-Schwesinger C.
Proc Natl Acad Sci U S A. 2019; 116(16):7963-7972

250

The intramembrane protease SPPL2c promotes male germ cell development by cleaving phospholamban.
Niemeyer J , Mentrup T , Heidasch R, Mueller S, Biswas U, Meyer R, Papadopoulou A, Dederer V, Haug-Kroeper M, Adamski V, Lüllmann-Rauch R, Bergmann M, Mayerhofer A, Saftig P, Wennemuth G, Jessberger R, Fluhrer R, Lichtenthaler S, Lemberg M, Schröder B.
EMBO R. 2019; 20(3):e46449

249

The Lysosomal Protein Arylsulfatase B Is a Key Enzyme Involved in Skeletal Turnover.
Pohl S, Angermann A, Jeschke A, Hendrickx G, Yorgan TA, Makrypidi-Fraune G, Steigert A, Kuehn SC, Rolvien T, Schweizer M, Koehne T, Neven M, Winter O, Velho RV, Albers J, Streichert T, Pestka JM, Baldauf C, Breyer S, Stuecker R, Muschol N, Cox TM, Saftig P, Paganini C, Rossi A, Amling M, Braulke T, Schinke T.
J Bone Miner Res. 2018 Dec;33(12):2186-2201

248

PLD3 and spinocerebellar ataxia.
Gonzalez AC, Stroobants S, Reisdorf P, Gavin AL, Nemazee D, Schwudke D, D'Hooge R, Saftig P, Damme M.
Brain. 2018 Nov 1;141(11):e78

247

In vivo regulation of the A disintegrin and metalloproteinase 10 (ADAM10) by the tetraspanin 15.
Seipold L, Altmeppen H, Koudelka T, Tholey A, Kasparek P, Sedlacek R, Schweizer M, Bär J, Mikhaylova M, Glatzel M, Saftig P.
Cell Mol Life Sci. 2018 Sep;75(17):3251-3267

246

Structural and mechanistic aspects influencing the ADAM10-mediated shedding of the prion protein.
Linsenmeier L, Mohammadi B, Wetzel S, Puig B, Jackson WS, Hartmann A, Uchiyama K, Sakaguchi S, Endres K, Tatzelt J, Saftig P, Glatzel M, Altmeppen HC.
Mol Neurodegener. 2018 Apr 6;13(1):18

245

Unconventional Trafficking of Mammalian Phospholipase D3 to Lysosomes.
Gonzalez AC, Schweizer M, Jagdmann S, Bernreuther C, Reinheckel T, Saftig P, Damme M.
Cell Rep. 2018 Jan 23;22(4):1040-1053

244

Lysosomal integral membrane protein-2 as a phospholipid receptor revealed by biophysical and cellular studies.
Conrad KS, Cheng TW, Ysselstein D, Heybrock S, Hoth LR, Chrunyk BA, Am Ende CW, Krainc D, Schwake M, Saftig P, Liu S, Qiu X, Ehlers MD.
Nat Commun. 2017 Dec 4;8(1):1908

243

Long-term enzyme replacement therapy improves neurocognitive functioning and hippocampal synaptic plasticity in immune-tolerant alpha-mannosidosis mice.
Stroobants S, Damme M, Van der Jeugd A, Vermaercke B, Andersson C, Fogh J, Saftig P, Blanz J, D'Hooge R.
Neurobiol Dis. 2017 Oct;106:255-268

242

Absence of RNase H2 triggers generation of immunogenic micronuclei removed by autophagy.
Bartsch K, Knittler K, Borowski C, Rudnik S, Damme M, Aden K, Spehlmann ME, Frey N, Saftig P, Chalaris A, Rabe B.
Hum Mol Genet. 2017 Oct 15;26(20):3960-3972

241

Intermittent Fasting Preserves Beta-Cell Mass in Obesity-induced Diabetes via the Autophagy-Lysosome Pathway.
Liu H, Javaheri A, Godar RJ, Murphy J, Ma X, Rohatgi N, Mahadevan J, Hyrc K, Saftig P, Marshall C, McDaniel ML, Remedi MS, Razani B, Urano F, Diwan A.
Autophagy. 2017;13(11):1952-1968

240

Progranulin functions as a cathepsin D chaperone to stimulate axonal outgrowth in vivo.
Beel S, Moisse M, Damme M, De Muynck L, Robberecht W, Van Den Bosch L, Saftig P, Van Damme P.
Hum Mol Genet. 2017 Aug 1;26(15):2850-2863

239

The Influence of MHC Class II on B Cell Defects Induced by Invariant Chain/CD74 N-Terminal Fragments.
Schneppenheim J, Loock AC, Hüttl S, Schweizer M, Lüllmann-Rauch R, Oberg HH, Arnold P, Lehmann CHK, Dudziak D, Kabelitz D, Lucius R, Lennon-Duménil AM, Saftig P, Schröder B.
J Immunol. 2017 Jul 1;199(1):172-185

238

Haplodeficiency of Cathepsin D does not affect cerebral amyloidosis and autophagy in APP/PS1 transgenic mice.
Cheng S, Wani WY, Hottman DA, Jeong A, Cao D, LeBlanc KJ, Saftig P, Zhang J, Li L.
J Neurochem. 2017 Jul;142(2):297-304

237

Functional characterization of the lysosomal membrane protein TMEM192 in mice.
Nguyen TL, Schneppenheim J, Rudnik S, Lüllmann-Rauch R, Bernreuther C, Hermans-Borgmeyer I, Glatzel M, Saftig P, Schröder B.
Oncotarget. 2017 Jul 4;8(27):43635-43652

236

Disruption of the vacuolar-type H+-ATPase complex in liver causes MTORC1-independent accumulation of autophagic vacuoles and lysosomes.
Kissing S, Rudnik S, Damme M, Lüllmann-Rauch R, Ichihara A, Kornak U, Eskelinen EL, Jabs S, Heeren J, De Brabander JK, Haas A, Saftig P.
Autophagy. 2017 Apr 3;13(4):670-685

235

Tetraspanin CD63 controls basolateral sorting of organic cation transporter 2 in renal proximal tubules.
Schulze U, Brast S, Grabner A, Albiker C, Snieder B, Holle S, Schlatter E, Schröter R, Pavenstädt H, Herrmann E, Lambert C, Spoden GA, Florin L, Saftig P, Ciarimboli G.
FASEB J. 2017 Apr;31(4):1421-1433

234

Sequestration of cholesterol within the host late endocytic pathway restricts liver-stage Plasmodium development.
Petersen W, Stenzel W, Silvie O, Blanz J, Saftig P, Matuschewski K, Ingmundson A.
Mol Biol Cell. 2017 Mar 15;28(6):726-735

233

Quantitative proteome analysis of mouse liver lysosomes provides evidence for mannose 6-phosphate-independent targeting mechanisms of acid hydrolases in mucolipidosis II.
Markmann S, Krambeck S, Hughes CJ, Mirzaian M, Aerts JM, Saftig P, Schweizer M, Vissers JC, Braulke T, Damme M.
Mol Cell Proteomics. 2017 Mar;16(3):438-450

232

Tetraspanin 3: A central endocytic membrane component regulating the expression of ADAM10, presenilin and the amyloid precursor protein.
Seipold L, Damme M, Prox J, Rabe B, Kasparek P, Sedlacek R, Altmeppen H, Willem M, Boland B, Glatzel M, Saftig P.
Biochim Biophys Acta Mol Cell Res. 2017 Jan;1864(1):217-230

231

Serum Starvation Induces BACE1 Processing and Secretion.
Stavropoulou AV, Mavrofrydi O, Saftig P, Efthimiopoulos S.
Curr Alzheimer Res. 2017;14(4):453-459

230

Pancreatic Premalignant Lesions Secrete Tissue Inhibitor of Metalloproteinases-1, Which Activates Hepatic Stellate Cells Via CD63 Signaling to Create a Premetastatic Niche in the Liver.
Grünwald B, Harant V, Schaten S, Frühschütz M, Spallek R, Höchst B, Stutzer K, Berchtold S, Erkan M, Prokopchuk O, Martignoni M, Esposito I, Heikenwalder M, Gupta A, Siveke J, Saftig P, Knolle P, Wohlleber D, Krüger A.
Gastroenterology. 2016 Nov;151(5):1011-1024.e7

229

Dissecting the role of ADAM10 as a mediator of S. aureus α-toxin action.
von Hoven G, Rivas AJ, Neukirch C, Klein S, Hamm C, Qin Q, Meyenburg M, Füser S, Saftig P, Hellmann N, Postina R, Husmann M.
Biochem J. 2016 Jul 1;473(13):1929-40

228

Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition)
Klionsky DJ, ..., Saftig P, ..., Zughaier SM.
Autophagy. 2016;12(1):1-222

227

ADAM17 controls IL-6 signaling by cleavage of the murine IL-6Rα from the cell surface of leukocytes during inflammatory responses.
Yan I, Schwarz J, Lücke K, Schumacher N, Schumacher V, Schmidt S, Rabe B, Saftig P, Donners M, Rose-John S, Mittrücker HW, Chalaris A.
J Leukoc Biol. 2016 May;99(5):749-60

226

Substrate determinants of signal peptide peptidase-like 2a (SPPL2a)-mediated intramembrane proteolysis of the invariant chain CD74.
Hüttl S, Helfrich F, Mentrup T, Held S, Fukumori A, Steiner H, Saftig P, Fluhrer R, Schröder B.
Biochem J. 2016 May 15;473(10):1405-22

225

Cathepsin K Deficiency Suppresses Disuse-Induced Bone Loss.
Moriya S, Izu Y, Arayal S, Kawasaki M, Hata K, Pawaputanon Na Mahasarakhahm C, Izumi Y, Saftig P, Kaneko K, Noda M, Ezura Y.
J Cell Physiol. 2016 May;231(5):1163-70

224

Characterization of the complex formed by β-glucocerebrosidase and the lysosomal integral membrane protein type-2.
Zunke F, Andresen L, Wesseler S, Groth J, Arnold P, Rothaug M, Mazzulli JR, Krainc D, Blanz J, Saftig P, Schwake M.
Proc Natl Acad Sci U S A. 2016 Apr 5;113(14):3791-6

223

Impaired Lysosomal Integral Membrane Protein 2-dependent Peroxiredoxin 6 delivery to lamellar bodies accounts for altered alveolar phospholipid content in Adaptor Protein-3-deficient pearl mice.
Kook S, Wang P, Young L, Schwake M, Saftig P, Weng X, Meng Y, Neculai D, Marks MS, Gonzales L, Beers MF, Guttentag S.
J Biol Chem. 2016 Apr 15;291(16):8414-27

222

A disintegrin and metalloprotease 10 (ADAM10) is a central regulator of murine liver tissue homeostasis.
Müller M, Wetzel S, Köhn-Gaone J, Chalupsky K, Lüllmann-Rauch R, Barikbin R, Bergmann J, Wöhner B, Zbodakova O, Leuschner I, Martin G, Tiegs G, Rose-John S, Sedlacek R, Tirnitz-Parker JE, Saftig P, Schmidt-Arras D.
Oncotarget. 2016 Apr 5;7(14):17431-41

221

LAMP proteins account for the maturation delay during the establishment of the Coxiella burnetii-containing vacuole.Schulze-Luehrmann J, Eckart RA, Ölke M, Saftig P, Liebler-Tenorio E, Lührmann A.
Cell Microbiol. 2016 Feb;18(2):181-94

220

Systematic substrate identification indicates a central role for the metalloprotease ADAM10 in axon targeting and synapse function.
Kuhn PH, Colombo AV, Schusser B, Dreymueller D, Wetzel S, Schepers U, Herber J, Ludwig A, Kremmer E, Montag D, Müller U, Schweizer M, Saftig P, Bräse S, Lichtenthaler SF.
Elife. 2016 Jan 23;5:e12748

219

Mannose 6-phosphate-independent Lysosomal Sorting of LIMP-2.
Blanz J, Zunke F, Markmann S, Damme M, Braulke T, Saftig P, Schwake M.
Traffic. 2015 Oct;16(10):1127-36

218

Chronic enzyme replacement therapy ameliorates neuropathology in alpha-mannosidosis mice.
Damme D, Stroobants S, Lüdemann M, Rothaug M, Lüllmann-Rauch R, Beck HC, Ericsson A, Andersson C, Fogh J, D’Hooge R, Saftig P, Blanz J.
Ann Clin Transl Neurol. 2015 Sep 19;2(11):987-1001

217

Repetitive stimulation of autophagy-lysosome machinery by intermittent fasting preconditions the myocardium to ischemia-reperfusion injury.
Godar RJ, Ma X, Liu H, Murphy JT, Weinheimer CJ, Kovacs A, Crosby SD, Saftig P, Diwan A.
Autophagy. 2015;11(9):1537-60

216

Processing of CD74 by the Intramembrane Protease SPPL2a Is Critical for B Cell Receptor Signaling in Transitional B Cells.
Hüttl S, Kläsener K, Schweizer M, Schneppenheim J, Oberg HH, Kabelitz D, Reth M, Saftig P, Schröder B.
J Immunol. 2015 Aug 15;195(4):1548-63

215

TIMP-1 signaling via CD63 triggers granulopoiesis and neutrophilia in mice.
Kobuch J, Cui H, Grünwald B, Saftig P, Knolle PA, Krüger A.
Haematologica. 2015 Aug;100(8):1005-13

214

A cell-based assay reveals nuclear translocation of intracellular domains released by SPPL proteases.
Mentrup, T., Häsler,R., Fluhrer, R., Saftig, P., Schröder, B.
Traffic. 2015 Aug;16(8):871-92

213

Vacuolar ATPase in Phagosome-Lysosome Fusion.
Kissing S, Hermsen C, Repnik U, Nesset CK, von Bargen K, Griffiths G, Ichihara A, Lee BS, Schwake M, De Brabander J, Haas A, Saftig P.
J Biol Chem. 2015 May 29;290(22):14166-80

212

Myeloid A Disintegrin and Metalloproteinase Domain 10 Deficiency Modulates Atherosclerotic Plaque Composition by Shifting the Balance from Inflammation toward Fibrosis.
van der Vorst EP, Jeurissen M, Wolfs IM, Keijbeck A, Theodorou K, Wijnands E, Schurgers L, Weber S, Gijbels MJ, Hamers AA, Dreymueller D, Rose-John S, de Winther MP, Ludwig A, Saftig P, Biessen EA, Donners MM.
Am J Pathol. 2015 Apr;185(4):1145-55

211

The sheddase ADAM10 is a potent modulator of prion disease.
Altmeppen HC, Prox J, Krasemann S, Puig B, Kruszewski K, Dohler F, Bernreuther C, Hoxha A, Linsenmeier L, Sikorska B, Liberski PP, Bartsch U, Saftig P, Glatzel M.
Elife. 2015 Feb 5;4:e04260

210

β-Secretase BACE1 Regulates Hippocampal and Reconstituted M-Currents in a β-Subunit-Like Fashion.
Hessler S, Zheng F, Hartmann S, Rittger A, Lehnert S, Völkel M, Nissen M, Edelmann E, Saftig P, Schwake M, Huth T, Alzheimer C.
J Neurosci. 2015 Feb 25;35(8):3298-311

209

Lysosomal integral membrane protein type-2 (LIMP-2/SCARB2) is a substrate of cathepsin-F, a cysteine protease mutated in type-B-Kufs-disease.
Peters J, Rittger A, Weisner R, Knabbe J, Zunke F, Rothaug M, Damme M, Berkovic SF, Blanz J, Saftig P, Schwake M.
Biochem Biophys Res Commun. 2015 Feb 13;457(3):334-40

208

LAMP-2 deficiency leads to hippocampal dysfunction but normal clearance of neuronal substrates of chaperone-mediated autophagy in a mouse model for Danon disease.
Rothaug M, Stroobants S, Schweizer M, Peters J, Zunke F, Allerding M, D’Hooge R, Saftig P, Blanz J.
Acta Neuropathol Commun. 2015 Jan 31;3:6

207

Cathepsin D deficiency induces oxidative damage in brain pericytes and impairs the blood-brain barrier.
Okada R, Wu Z, Zhu A, Ni J, Zhang JQ, Yoshimine Y, Peters C, Saftig P, Nakanishi H.
Mol Cell Neurosci. 2015 Jan;64:51-60

206

LIMP-2 expression is critical for β-glucocerebrosidase activity and α-synuclein clearance.
Rothaug M, Zunke F, Mazzulli JR, Schweizer M, Altmeppen H, Lüllmann-Rauch R, Kallemeijn WW, Gaspar P, Aerts JM, Glatzel M, Saftig P, Krainc D, Schwake M, Blanz J.
Proc Natl Acad Sci U S A. 2014 Oct 28;111(43):15573-8

205

High susceptibility to fatty liver disease in two-pore channel 2-deficient mice.
Grimm C, Holdt LM, Chen CC, Hassan S, Müller C, Jörs S, Cuny H, Kissing S, Schröder B, Butz E, Northoff B, Castonguay J, Luber CA, Moser M, Spahn S, Lüllmann-Rauch R, Fendel C, Klugbauer N, Griesbeck O, Haas A, Mann M, Bracher F, Teupser D, Saftig P, Biel M, Wahl-Schott C.
Nat Commun. 2014 Aug 21;5:4699

204

Signal-peptide-peptidase-like 2a is required for CD74 intramembrane proteolysis in human B cells.
Schneppenheim J, Hüttl S, Kruchen A, Fluhrer R, Müller I, Saftig P, Schneppenheim R, Martin CL, Schröder B.
Biochem Biophys Res Commun. 2014 Aug 15;451(1):48-53

203

Regulated Proteolysis of NOTCH2 and NOTCH3 Receptors by ADAM10 and Presenilins.
Groot AJ, Habets R, Yahyanejad S, Hodin CM, Reiss K, Saftig P, Theys J, Vooijs M.
Mol Cell Biol. 2014 Aug;34(15):2822-32

202

Virus entry. Lassa virus entry requires a trigger-induced receptor switch.
Jae LT, Raaben M, Herbert AS, Kuehne AI, Wirchnianski AS, Soh TK, Stubbs SH, Janssen H, Damme M, Saftig P, Whelan SP, Dye JM, Brummelkamp TR.
Science. 2014 Jun 27;344(6191):1506-10

201

Leukocytes require ADAM10 but not ADAM17 for their migration and inflammatory recruitment into the alveolar space.
Pruessmeyer J, Hess FM, Alert H, Groth E, Pasqualon T, Schwarz N, Nyamoya S, Kollert J, van der Vorst E, Donners M, Martin C, Uhlig S, Saftig P, Dreymueller D, Ludwig A.
Blood. 2014 Jun 26;123(26):4077-88

200

ADAM metalloproteases promote a developmental switch in responsiveness to the axonal repellant Sema3A.
Romi E, Gokhman I, Wong E, Antonovsky N, Ludwig A, Sagi I, Saftig P, Tessier-Lavigne M, Yaron A.
Nat Commun. 2014 Jun 5;5:4058

199

The intramembrane proteases signal Peptide peptidase-like 2a and 2b have distinct functions in vivo.Schneppenheim J, Hüttl S, Mentrup T, Lüllmann-Rauch R, Rothaug M, Engelke M, Dittmann K, Dressel R, Araki M, Araki K, Wienands J, Fluhrer R, Saftig P, Schröder B.
Mol Cell Biol. 2014 Apr;34(8):1398-411

198

The endolysosomal cysteine cathepsins L and K are involved in macrophage-mediated clearance of Staphylococcus aureus and the concomitant cytokine induction.
Müller S, Faulhaber A, Sieber C, Pfeifer D, Hochberg T, Gansz M, Deshmukh SD, Dauth S, Brix K, Saftig P, Peters C, Henneke P, Reinheckel T.
FASEB J. 2014 Jan;28(1):162-75

197

Regulation of adult hematopoiesis by the a disintegrin and metalloproteinase 10 (ADAM10).
Weber S, Wetzel S, Prox J, Lehmann T, Schneppenheim J, Donners M, Saftig P.
Biochem Biophys Res Commun. 2013 Dec 13;442(3-4):234-41

196

Structure of LIMP-2 provides functional insights with implications for SR-BI and CD36.
Neculai D, Schwake M, Ravichandran M, Zunke F, Collins RF, Peters J, Neculai M, Plumb J, Loppnau P, Pizarro JC, Seitova A, Trimble WS, Saftig P, Grinstein S, Dhe-Paganon S.
Nature. 2013 Dec 5;504(7478):172-6

195

Enzyme replacement therapy for alpha-mannosidosis: 12 months follow-up of a single centre, randomised, multiple dose study.
Borgwardt L, Dali CI, Fogh J, Månsson JE, Olsen KJ, Beck HC, Nielsen KG, Nielsen LH, Olsen SO, Riise Stensland HM, Nilssen O, Wibrand F, Thuesen AM, Pearl T, Haugsted U, Saftig P, Blanz J, Jones SA, Tylki-Szymanska A, Guffon-Fouiloux N, Beck M, Lund AM.
J Inherit Metab Dis. 2013 Nov;36(6):1015-24

194

Epidermal ADAM17 is dispensable for notch activation.
Groot AJ, Cobzaru C, Weber S, Saftig P, Blobel CP, Kopan R, Vooijs M, Franzke CW.
J Invest Dermatol. 2013 Sep;133(9):2286-8

193

The tetraspanin CD63 is required for efficient IgE-mediated mast cell degranulation and anaphylaxis.
Kraft S, Jouvin MH, Kulkarni N, Kissing S, Morgan ES, Dvorak AM, Schröder B, Saftig P, Kinet JP.
J Immunol. 2013 Sep 15;191(6):2871-8

192

Surface CD107a/LAMP-1 protects natural killer cells from degranulation-associated damage.
Cohnen A, Chiang SC, Stojanovic A, Schmidt H, Claus M, Saftig P, Janßen O, Cerwenka A, Bryceson YT, Watzl C.
Blood. 2013 Aug 22;122(8):1411-8

191

Postnatal disruption of the disintegrin/metalloproteinase ADAM10 in brain causes epileptic seizures, learning deficits, altered spine morphology, and defective synaptic functions.
Prox J, Bernreuther C, Altmeppen H, Grendel J, Glatzel M, D’Hooge R, Stroobants S, Ahmed T, Balschun D, Willem M, Lammich S, Isbrandt D, Schweizer M, Horré K, De Strooper B, Saftig P.
J Neurosci. 2013 Aug 7;33(32):12915-28, 12928a

190

Bace1 and Neuregulin-1 cooperate to control formation and maintenance of muscle spindles.
Cheret C, Willem M, Fricker FR, Wende H, Wulf-Goldenberg A, Tahirovic S, Nave KA, Saftig P, Haass C, Garratt AN, Bennett DL, Birchmeier C.
EMBO J. 2013 Jul 17;32(14):2015-28

189

The intramembrane protease SPPL2A is critical for tooth enamel formation.
Bronckers AL, Gueneli N, Lüllmann-Rauch R, Schneppenheim J, Moraru AP, Himmerkus N, Bervoets TJ, Fluhrer R, Everts V, Saftig P, Schröder B.
J Bone Miner Res. 2013 Jul;28(7):1622-30

188

Natural history of alpha mannosidosis a longitudinal study.
Beck M, Olsen KJ, Wraith JE, Zeman J, Michalski JC, Saftig P, Fogh J, Malm D.
Orphanet J Rare Dis. 2013 Jun 20;8:88

187

Parallel regulation of renin and lysosomal integral membrane protein 2 in renin-producing cells: further evidence for a lysosomal nature of renin secretory vesicles.
Schmid J, Oelbe M, Saftig P, Schwake M, Schweda F.
Pflugers Arch. 2013 Jun;465(6):895-905

186

Cathepsin F mutations cause Type B Kufs disease, an adult-onset neuronal ceroid lipofuscinosis.
Smith KR, Dahl HH, Canafoglia L, Andermann E, Damiano J, Morbin M, Bruni AC, Giaccone G, Cossette P, Saftig P, Grötzinger J, Schwake M, Andermann F, Staropoli JF, Sims KB, Mole SE, Franceschetti S, Alexander NA, Cooper JD, Chapman HA, Carpenter S, Berkovic SF, Bahlo M.
Hum Mol Genet. 2013 Apr 1;22(7):1417-23

185

Extracellular cathepsin K exerts antimicrobial activity and is protective against chronic intestinal inflammation in mice.
Sina C, Lipinski S, Gavrilova O, Aden K, Rehman A, Till A, Rittger A, Podschun R, Meyer-Hoffert U, Haesler R, Midtling E, Pütsep K, McGuckin MA, Schreiber S, Saftig P*, Rosenstiel P*.
Gut. 2013 Apr;62(4):520-30 *: corresponding author

184

Cellular mechanotransduction relies on tension-induced and chaperone-assisted autophagy.
Ulbricht A, Eppler FJ, Tapia VE, van der Ven PF, Hampe N, Hersch N, Vakeel P, Stadel D, Haas A, Saftig P, Behrends C, Fürst DO, Volkmer R, Hoffmann B, Kolanus W, Höhfeld J.
Curr Biol. 2013 Mar 4;23(5):430-5

183

Differences in expression patterns of cathepsin C/dipeptidyl peptidase I in normal, pathological and aged mouse central nervous system.
Koike M, Shibata M, Ezaki J, Peters C, Saftig P, Kominami E, Uchiyama Y.
Eur J Neurosci. 2013 Mar;37(5):816-30

182

The intramembrane protease SPPL2a promotes B cell development and controls endosomal traffic by cleavage of the invariant chain.
Schneppenheim J, Dressel R, Hüttl S, Lüllmann-Rauch R, Engelke M, Dittmann K, Wienands J, Eskelinen EL, Hermans-Borgmeyer I, Fluhrer R, Saftig P, Schröder B.
J Exp Med. 2013 Jan 14;210(1):41-58

181

Sensitivity to lysosome-dependent cell death is directly regulated by lysosomal cholesterol content.
Appelqvist H, Sandin L, Björnström K, Saftig P, Garner B, Ollinger K, Kågedal K.
PLoS One. 2012;7(11):e50262

180

Effects of cathepsin K deficiency on intercellular junction proteins, luminal mucus layers, and extracellular matrix constituents in the mouse colon.
Arampatzidou M, Schütte A, Hansson GC, Saftig P, Brix K.
Biol Chem. 2012 Dec;393(12):1391-403

179

Activity-dependent proteolytic cleavage of neuroligin-1.
Suzuki K, Hayashi Y, Nakahara S, Kumazaki H, Prox J, Horiuchi K, Zeng M, Tanimura S, Nishiyama Y, Osawa S, Sehara-Fujisawa A, Saftig P, Yokoshima S, Fukuyama T, Matsuki N, Koyama R, Tomita T, Iwatsubo T.
Neuron. 2012 Oct 18;76(2):410-22

178

Adam10 haploinsufficiency causes freckle-like macules in Hairless mice.
Tharmarajah G, Faas L, Reiss K, Saftig P, Young A, Van Raamsdonk CD.
Pigment Cell Melanoma Res. 2012 Sep;25(5):555-65

177

β-Secretase (BACE1) inhibition causes retinal pathology by vascular dysregulation and accumulation of age pigment.
Cai J, Qi X, Kociok N, Skosyrski S, Emilio A, Ruan Q, Han S, Liu L, Chen Z, Bowes Rickman C, Golde T, Grant MB, Saftig P, Serneels L, de Strooper B, Joussen AM, Boulton ME.
EMBO Mol Med. 2012 Sep;4(9):980-91

176

The lysosomal polypeptide transporter TAPL is stabilized by interaction with LAMP-1 and LAMP-2.
Demirel Ö, Jan I, Wolters D, Blanz J, Saftig P, Tampé R, Abele R.
J Cell Sci. 2012 Sep 15;125(Pt 18):4230-40

175

Tetraspanin15 regulates cellular trafficking and activity of the ectodomain sheddase ADAM10.
Prox J, Willenbrock M, Weber S, Lehmann T, Schmidt-Arras D, Schwanbeck R, Saftig P, Schwake M.
Cell Mol Life Sci. 2012 Sep;69(17):2919-32

174

A critical histidine residue within LIMP-2 mediates pH sensitive binding to its ligand β-glucocerebrosidase.
Zachos C, Blanz J, Saftig P, Schwake M.
Traffic. 2012 Aug;13(8):1113-23

173

Notch-RBP-J signaling regulates the transcription factor IRF8 to promote inflammatory macrophage polarization.
Xu H, Zhu J, Smith S, Foldi J, Zhao B, Chung AY, Outtz H, Kitajewski J, Shi C, Weber S, Saftig P, Li Y, Ozato K, Blobel CP, Ivashkiv LB, Hu X.
Nat Immunol. 2012 May 20;13(7):642-50

172

Physiological functions of the amyloid precursor protein secretases ADAM10, BACE1, and presenilin.
Prox J, Rittger A, Saftig P.
Exp Brain Res. 2012 Apr;217(3-4):331-41

171

Mannose 6 dephosphorylation of lysosomal proteins mediated by acid phosphatases Acp2 and Acp5.
Makrypidi G, Damme M, Müller-Loennies S, Trusch M, Schmidt B, Schlüter H, Heeren J, Lübke T, Saftig P, Braulke T.
Mol Cell Biol. 2012 Feb;32(4):774-82

170

Signal-peptide-peptidase-like 2a (SPPL2a) is targeted to lysosomes/late endosomes by a tyrosine motif in its C-terminal tail.
Behnke J, Schneppenheim J, Koch-Nolte F, Haag F, Saftig P, Schröder B.
FEBS Lett. 2011 Oct 3;585(19):2951-7

169

The tetraspanin CD63 regulates ESCRT-independent and -dependent endosomal sorting during melanogenesis.
van Niel G, Charrin S, Simoes S, Romao M, Rochin L, Saftig P, Marks MS, Rubinstein E, Raposo G.
Dev Cell. 2011 Oct 18;21(4):708-21

168

The Swedish APP mutation alters the effect of genetically reduced BACE1 expression on the APP processing.
Rabe S, Reichwald J, Ammaturo D, de Strooper B, Saftig P, Neumann U, Staufenbiel M.
J Neurochem. 2011 Oct;119(1):231-9

167

CD63 is an essential cofactor to leukocyte recruitment by endothelial P-selectin.
Doyle EL, Ridger V, Ferraro F, Turmaine M, Saftig P, Cutler DF.
Blood. 2011 Oct 13;118(15):4265-73

166

Redundancy and specificity of the metalloprotease system mediating oncogenic NOTCH1 activation in T-ALL.Sulis ML, Saftig P, Ferrando AA.
Leukemia. 2011 Oct;25(10):1564-9

165

Disrupted in renal carcinoma 2 (DIRC2), a novel transporter of the lysosomal membrane, is proteolytically processed by cathepsin L.Savalas LR, Gasnier B, Damme M, Lübke T, Wrocklage C, Debacker C, Jézégou A, Reinheckel T, Hasilik A, Saftig P, Schröder B.
Biochem J. 2011 Oct 1;439(1):113-28

164

Neuronal brain-derived neurotrophic factor is synthesized in excess, with levels regulated by sortilin-mediated trafficking and lysosomal degradation.
Evans SF, Irmady K, Ostrow K, Kim T, Nykjaer A, Saftig P, Blobel C, Hempstead BL.
J Biol Chem. 2011 Aug 26;286(34):29556-67

163

Cathepsin K deficiency in mice induces structural and metabolic changes in the central nervous system that are associated with learning and memory deficits.
Dauth S, Sîrbulescu RF, Jordans S, Rehders M, Avena L, Oswald J, Lerchl A, Saftig P, Brix K.
BMC Neurosci. 2011 Jul 27;12:74

162

Deletion of Adam10 in endothelial cells leads to defects in organ-specific vascular structures.
Glomski K, Monette S, Manova K, De Strooper B, Saftig P, Blobel CP.
Blood. 2011 Jul 28;118(4):1163-74

161

Tubular proteinuria in mice and humans lacking the intrinsic lysosomal protein SCARB2/Limp-2.
Desmond MJ, Lee D, Fraser SA, Katerelos M, Gleich K, Martinello P, Li YQ, Thomas MC, Michelucci R, Cole AJ, Saftig P, Schwake M, Stapleton D, Berkovic SF, Power DA.
Am J Physiol Renal Physiol. 2011 Jun;300(6):F1437-47

160

Acid β-glucosidase mutants linked to Gaucher disease, Parkinson disease, and Lewy body dementia alter α-synuclein processing.
Cullen V, Sardi SP, Ng J, Xu YH, Sun Y, Tomlinson JJ, Kolodziej P, Kahn I, Saftig P, Woulfe J, Rochet JC, Glicksman MA, Cheng SH, Grabowski GA, Shihabuddin LS, Schlossmacher MG.
Ann Neurol. 2011 Jun;69(6):940-53

159

Lack of a-disintegrin-and-metalloproteinase ADAM10 leads to intracellular accumulation and loss of shedding of the cellular prion protein in vivo.Altmeppen HC, Prox J, Puig B, Kluth MA, Bernreuther C, Thurm D, Jorissen E, Petrowitz B, Bartsch U, De Strooper B, Saftig P, Glatzel M.
Mol Neurodegener. 2011 May 27;6:36

158

The effect of cathepsin K deficiency on airway development and TGF-β1 degradation.
Zhang D, Leung N, Weber E, Saftig P, Brömme D.
Respir Res. 2011 May 31;12(1):72

157

Two dileucine motifs mediate late endosomal/lysosomal targeting of transmembrane protein 192 (TMEM192) and a C-terminal cysteine residue is responsible for disulfide bond formation in TMEM192 homodimers.
Behnke J, Eskelinen EL, Saftig P, Schröder B.
Biochem J. 2011 Mar 1;434(2):219-31

156

β-Site APP-cleaving enzyme 1 (BACE1) cleaves cerebellar Na⁺ channel β4-subunit and promotes Purkinje cell firing by slowing the decay of resurgent Na⁺ current.
Huth T, Rittger A, Saftig P, Alzheimer C.
Pflugers Arch. 2011 Mar;461(3):355-71

155

Sphingolipid storage affects autophagic metabolism of the amyloid precursor protein and promotes Abeta generation.
Tamboli IY, Hampel H, Tien NT, Tolksdorf K, Breiden B, Mathews PM, Saftig P, Sandhoff K, Walter J.
J Neurosci. 2011 Feb 2;31(5):1837-49

154

Role for LAMP-2 in endosomal cholesterol transport.
Schneede A, Schmidt CK, Hölttä-Vuori M, Heeren J, Willenborg M, Blanz J, Domanskyy M, Breiden B, Brodesser S, Landgrebe J, Sandhoff K, Ikonen E, Saftig P, Eskelinen EL.
J Cell Mol Med. 2011 Feb;15(2):280-95

153

LIMP-2 links late phagosomal trafficking with the onset of the innate immune response to Listeria monocytogenes: a role in macrophage activation.
Carrasco-Marín E, Fernández-Prieto L, Rodriguez-Del Rio E, Madrazo-Toca F, Reinheckel T, Saftig P, Alvarez-Dominguez C.
J Biol Chem. 2011 Feb 4;286(5):3332-41

152

Cerebellar alterations and gait defects as therapeutic outcome measures for enzyme replacement therapy in α-mannosidosis.
Damme M, Stroobants S, Walkley SU, Lüllmann-Rauch R, D’Hooge R, Fogh J, Saftig P, Lübke T, Blanz J.
J Neuropathol Exp Neurol. 2011 Jan;70(1):83-94

151

ADAM17 is regulated by a rapid and reversible mechanism that controls access to its catalytic site.
Le Gall SM, Maretzky T, Issuree PD, Niu XD, Reiss K, Saftig P, Khokha R, Lundell D, Blobel CP.
J Cell Sci. 2010 Nov 15;123(Pt 22):3913-22

150

Critical role of the disintegrin metalloprotease ADAM17 for intestinal inflammation and regeneration in mice.
Chalaris A, Adam N, Sina C, Rosenstiel P, Lehmann-Koch J, Schirmacher P, Hartmann D, Cichy J, Gavrilova O, Schreiber S, Jostock T, Matthews V, Häsler R, Becker C, Neurath MF, Reiss K, Saftig P, Scheller J, Rose-John S.
J Exp Med. 2010 Aug 2;207(8):1617-24

149

Stimulation of platelet-derived growth factor receptor beta (PDGFRbeta) activates ADAM17 and promotes metalloproteinase-dependent cross-talk between the PDGFRbeta and epidermal growth factor receptor (EGFR) signaling pathways.
Mendelson K, Swendeman S, Saftig P, Blobel CP.
J Biol Chem. 2010 Aug 6;285(32):25024-32

148

Activity-dependent alpha-cleavage of nectin-1 is mediated by a disintegrin and metalloprotease 10 (ADAM10).
Kim J, Lilliehook C, Dudak A, Prox J, Saftig P, Federoff HJ, Lim ST.
J Biol Chem. 2010 Jul 23;285(30):22919-26

147

CNS-expressed cathepsin D prevents lymphopenia in a murine model of congenital neuronal ceroid lipofuscinosis.
Shevtsova Z, Garrido M, Weishaupt J, Saftig P, Bähr M, Lühder F, Kügler S.
Am J Pathol. 2010 Jul;177(1):271-9

146

Molecular characterisation of 'transmembrane protein 192' (TMEM192), a novel protein of the lysosomal membrane.
Schröder B, Wrocklage C, Hasilik A, Saftig P.
Biol Chem. 2010 Jun;391(6):695-704

145

Molecular insights into mechanisms of intramembrane proteolysis through signal peptide peptidase (SPP).
Schröder B, Saftig P.
Biochem J. 2010 Apr 14;427(3):e1-3

144

The disintegrin/metalloproteinase ADAM10 is essential for the establishment of the brain cortex.
Jorissen E, Prox J, Bernreuther C, Weber S, Schwanbeck R, Serneels L, Snellinx A, Craessaerts K, Thathiah A, Tesseur I, Bartsch U, Weskamp G, Blobel CP, Glatzel M, De Strooper B, Saftig P.
Version 2. J Neurosci. 2010 Apr 7;30(14):4833-44

143

Cathepsin K gene disruption does not affect murine aneurysm formation.
Bai L, Beckers L, Wijnands E, Lutgens SP, Herías MV, Saftig P, Daemen MJ, Cleutjens K, Lutgens E, Biessen EA, Heeneman S.
Atherosclerosis. 2010 Mar;209(1):96-103

142

Disease-causing mutations within the lysosomal integral membrane protein type 2 (LIMP-2) reveal the nature of binding to its ligand beta-glucocerebrosidase.
Blanz J, Groth J, Zachos C, Wehling C, Saftig P, Schwake M.
Hum Mol Genet. 2010 Feb 15;19(4):563-72

141

Chaperone-assisted selective autophagy is essential for muscle maintenance.
Arndt V, Dick N, Tawo R, Dreiseidler M, Wenzel D, Hesse M, Fürst DO, Saftig P, Saint R, Fleischmann BK, Hoch M, Höhfeld J.
Curr Biol. 2010 Jan 26;20(2):143-8

140

Cathepsin B-mediated autophagy flux facilitates the anthrax toxin receptor 2-mediated delivery of anthrax lethal factor into the cytoplasm.
Ha SD, Ham B, Mogridge J, Saftig P, Lin S, Kim SO.
J Biol Chem. 2010 Jan 15;285(3):2120-9

139

Alcadein cleavages by amyloid beta-precursor protein (APP) alpha- and gamma-secretases generate small peptides, p3-Alcs, indicating Alzheimer disease-related gamma-secretase dysfunction.
Hata S, Fujishige S, Araki Y, Kato N, Araseki M, Nishimura M, Hartmann D, Saftig P, Fahrenholz F, Taniguchi M, Urakami K, Akatsu H, Martins RN, Yamamoto K, Maeda M, Yamamoto T, Nakaya T, Gandy S, Suzuki T.
J Biol Chem. 2009 Dec 25;284(52):36024-33

138

Cathepsin D is the primary protease for the generation of adenohypophyseal vasoinhibins: cleavage occurs within the prolactin secretory granules.
Cruz-Soto ME, Cosío G, Jeziorski MC, Vargas-Barroso V, Aguilar MB, Cárabez A, Berger P, Saftig P, Arnold E, Thebault S, Martínez de la Escalera G, Clapp C.
Endocrinology. 2009 Dec;150(12):5446-54

137

Glycosaminoglycan-mediated loss of cathepsin K collagenolytic activity in MPS I contributes to osteoclast and growth plate abnormalities.
Wilson S, Hashamiyan S, Clarke L, Saftig P, Mort J, Dejica VM, Brömme D.
Am J Pathol. 2009 Nov;175(5):2053-62

136

Klotho is a substrate for alpha-, beta- and gamma-secretase.
Bloch L, Sineshchekova O, Reichenbach D, Reiss K, Saftig P, Kuro-o M, Kaether C.
FEBS Lett. 2009 Oct 6;583(19):3221-4

135

Cytoplasmic relaxation of active Eph controls ephrin shedding by ADAM10.Janes PW, Wimmer-Kleikamp SH, Frangakis AS, Treble K, Griesshaber B, Sabet O, Grabenbauer M, Ting AY, Saftig P, Bastiaens PI, Lackmann M.
PLoS Biol. 2009 Oct;7(10):e1000215

134

ADAM10, the rate-limiting protease of regulated intramembrane proteolysis of Notch and other proteins, is processed by ADAMS-9, ADAMS-15, and the gamma-secretase.
Tousseyn T, Thathiah A, Jorissen E, Raemaekers T, Konietzko U, Reiss K, Maes E, Snellinx A, Serneels L, Nyabi O, Annaert W, Saftig P, Hartmann D, De Strooper B.
J Biol Chem. 2009 Apr 24;284(17):11738-47

133

ADAMs 10 and 17 represent differentially regulated components of a general shedding machinery for membrane proteins such as transforming growth factor alpha, L-selectin, and tumor necrosis factor alpha.
Le Gall SM, Bobé P, Reiss K, Horiuchi K, Niu XD, Lundell D, Gibb DR, Conrad D, Saftig P, Blobel CP.
Mol Biol Cell. 2009 Mar;20(6):1785-94

132

Cathepsin D expression level affects alpha-synuclein processing, aggregation, and toxicity in vivo.
Cullen V, Lindfors M, Ng J, Paetau A, Swinton E, Kolodziej P, Boston H, Saftig P, Woulfe J, Feany MB, Myllykangas L, Schlossmacher MG, Tyynelä J.
Mol Brain. 2009 Feb 9;2:5

131

Deficiency of the tetraspanin CD63 associated with kidney pathology but normal lysosomal function.
Schröder J, Lüllmann-Rauch R, Himmerkus N, Pleines I, Nieswandt B, Orinska Z, Koch-Nolte F, Schröder B, Bleich M, Saftig P.
Mol Cell Biol. 2009 Feb;29(4):1083-94

130

Non-proteolytic effect of beta-site APP-cleaving enzyme 1 (BACE1) on sodium channel function.
Huth T, Schmidt-Neuenfeldt K, Rittger A, Saftig P, Reiss K, Alzheimer C.
Neurobiol Dis. 2009 Feb;33(2):282-9

129

Bone density, strength, and formation in adult cathepsin K (-/-) mice.
Pennypacker B, Shea M, Liu Q, Masarachia P, Saftig P, Rodan S, Rodan G, Kimmel D.
Bone. 2009 Feb;44(2):199-207

128

Cathepsin K activity-dependent regulation of osteoclast actin ring formation and bone resorption.
Wilson SR, Peters C, Saftig P, Brömme D.
J Biol Chem. 2009 Jan 23;284(4):2584-92

127

Reversal of peripheral and central neural storage and ataxia after recombinant enzyme replacement therapy in alpha-mannosidosis mice.
Blanz J, Stroobants S, Lüllmann-Rauch R, Morelle W, Lüdemann M, D’Hooge R, Reuterwall H, Michalski JC, Fogh J, Andersson C, Saftig P.
Hum Mol Genet. 2008 Nov 15;17(22):3437-45

126

A soluble form of the receptor for advanced glycation endproducts (RAGE) is produced by proteolytic cleavage of the membrane-bound form by the sheddase a disintegrin and metalloprotease 10 (ADAM10).
Raucci A, Cugusi S, Antonelli A, Barabino SM, Monti L, Bierhaus A, Reiss K, Saftig P, Bianchi ME.
FASEB J. 2008 Oct;22(10):3716-27

125

Role of cathepsin K in structural changes in brachiocephalic artery during progression of atherosclerosis in apoE-deficient mice.
Samokhin AO, Wong A, Saftig P, Brömme D.
Atherosclerosis. 2008 Sep;200(1):58-68

124

Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis.
Jabs S, Quitsch A, Käkelä R, Koch B, Tyynelä J, Brade H, Glatzel M, Walkley S, Saftig P, Vanier MT, Braulke T.
J Neurochem. 2008 Aug;106(3):1415-25

123

ADAM10-mediated E-cadherin release is regulated by proinflammatory cytokines and modulates keratinocyte cohesion in eczematous dermatitis.
Maretzky T, Scholz F, Köten B, Proksch E, Saftig P, Reiss K.
J Invest Dermatol. 2008 Jul;128(7):1737-46

122

ADAM10 regulates endothelial permeability and T-Cell transmigration by proteolysis of vascular endothelial cadherin.Schulz B, Pruessmeyer J, Maretzky T, Ludwig A, Blobel CP, Saftig P, Reiss K.
Circ Res. 2008 May 23;102(10):1192-201

121

Array-based gene discovery with three unrelated subjects shows SCARB2/LIMP-2 deficiency causes myoclonus epilepsy and glomerulosclerosis.
Berkovic SF, Dibbens LM, Oshlack A, Silver JD, Katerelos M, Vears DF, Lüllmann-Rauch R, Blanz J, Zhang KW, Stankovich J, Kalnins RM, Dowling JP, Andermann E, Andermann F, Faldini E, D’Hooge R, Vadlamudi L, Macdonell RA, Hodgson BL, Bayly MA, Savige J, Mulley JC, Smyth GK, Power DA, Saftig P, Bahlo M.
Am J Hum Genet. 2008 Mar;82(3):673-84

120

Cathepsin K-dependent toll-like receptor 9 signaling revealed in experimental arthritis.
Asagiri M, Hirai T, Kunigami T, Kamano S, Gober HJ, Okamoto K, Nishikawa K, Latz E, Golenbock DT, Aoki K, Ohya K, Imai Y, Morishita Y, Miyazono K, Kato S, Saftig P, Takayanagi H.
Science. 2008 Feb 1;319(5863):624-7

119

Beertsen W, Willenborg M, Everts V, Zirogianni A, Podschun R, Schröder B, Eskelinen EL, Saftig P. (2008)
Impaired phagosomal maturation in neutrophils leads to periodontitis in lysosomal-associated membrane protein-2 knockout mice.
J Immunol 180(1):475-82.

118

CathepsinK deficiency partially inhibits, but does not prevent, bone destructionin human tumor necrosis factor-transgenic mice.
Schurigt U, Hummel KM, Petrow PK, Gajda M, Stöckigt R, Middel P, Zwerina J, Janik T, Bernhardt R, Schüler S, Scharnweber D, Beckmann F, Saftig P, Kollias G, Schett G, Wiederanders B, Bräuer R.
Arthritis Rheum. 2008 Feb;58(2):422-34

117

Regulated intramembrane proteolysis of Bri2 (Itm2b) by ADAM10 and SPPL2a/SPPL2b.
Martin L, Fluhrer R, Reiss K, Kremmer E, Saftig P, Haass C.
J Biol Chem. 2008 Jan 18;283(3):1644-52

116

Synaptic changes in the thalamocortical system of cathepsin D-deficient mice: a model of human congenital neuronal ceroid-lipofuscinosis.
Partanen S, Haapanen A, Kielar C, Pontikis C, Alexander N, Inkinen T, Saftig P, Gillingwater TH, Cooper JD, Tyynelä J.
J Neuropathol Exp Neurol. 2008 Jan;67(1):16-29

115

LIMP-2 is a receptor for lysosomal mannose-6-phosphate-independent targeting of beta-glucocerebrosidase.
Reczek D, Schwake M, Schröder J, Hughes H, Blanz J, Jin X, Brondyk W, Van Patten S, Edmunds T, Saftig P.
Cell. 2007 Nov 16;131(4):770-83

114

Haematopoietic development and immunological function in the absence of cathepsin D.
Tulone C, Uchiyama Y, Novelli M, Grosvenor N, Saftig P, Chain BM.
BMC Immunol. 2007 Sep 26;8:22

113

Arrested maturation of Neisseria-containing phagosomes in the absence of the lysosome-associated membrane proteins, LAMP-1 and LAMP-2.
Binker MG, Cosen-Binker LI, Terebiznik MR, Mallo GV, McCaw SE, Eskelinen EL, Willenborg M, Brumell JH, Saftig P, Grinstein S, Gray-Owen SD.
Cell Microbiol. 2007 Sep;9(9):2153-66

112

Toll-like receptor 4-dependent contribution of the immune system to anticancer chemotherapy and radiotherapy.
Apetoh L, Ghiringhelli F, Tesniere A, Obeid M, Ortiz C, Criollo A, Mignot G, Maiuri MC, Ullrich E, Saulnier P, Yang H, Amigorena S, Ryffel B, Barrat FJ, Saftig P, Levi F, Lidereau R, Nogues C, Mira JP, Chompret A, Joulin V, Clavel-Chapelon F, Bourhis J, André F, Delaloge S, Tursz T, Kroemer G, Zitvogel L.
Nat Med. 2007 Sep;13(9):1050-9

111

Cathepsin K null mice show reduced adiposity during the rapid accumulation of fat stores.
Funicello M, Novelli M, Ragni M, Vottari T, Cocuzza C, Soriano-Lopez J, Chiellini C, Boschi F, Marzola P, Masiello P, Saftig P, Santini F, St-Jacques R, Desmarais S, Morin N, Mancini J, Percival MD, Pinchera A, Maffei M.
PLoS One. 2007 Aug 1;2(8):e683

110

Involvement of lysosomal storage-induced p38 MAP kinase activation in the overproduction of nitric oxide by microglia in cathepsin D-deficient mice.
Yamasaki R, Zhang J, Koshiishi I, Sastradipura Suniarti DF, Wu Z, Peters C, Schwake M, Uchiyama Y, Kira J, Saftig P, Utsumi H, Nakanishi H.
Mol Cell Neurosci. 2007 Aug;35(4):573-84

109

Regulated shedding of transmembrane chemokines by the disintegrin and metalloproteinase 10 facilitates detachment of adherent leukocytes.
Hundhausen C, Schulte A, Schulz B, Andrzejewski MG, Schwarz N, von Hundelshausen P, Winter U, Paliga K, Reiss K, Saftig P, Weber C, Ludwig A.
J Immunol. 2007 Jun 15;178(12):8064-72

108

Altered regulation of phosphatidylinositol 3-kinase signaling in cathepsin D-deficient brain.
Walls KC, Klocke BJ, Saftig P, Shibata M, Uchiyama Y, Roth KA, Shacka JJ.
Autophagy. 2007 May-Jun;3(3):222-9

107

ADAM10 regulates FasL cell surface expression and modulates FasL-induced cytotoxicity and activation-induced cell death.
Schulte M, Reiss K, Lettau M, Maretzky T, Ludwig A, Hartmann D, de Strooper B, Janssen O, Saftig P.
Cell Death Differ. 2007 May;14(5):1040-9

106

Lysosomal integral membrane protein 2 is a novel component of the cardiac intercalated disc and vital for load-induced cardiac myocyte hypertrophy.
Schroen B, Leenders JJ, van Erk A, Bertrand AT, van Loon M, van Leeuwen RE, Kubben N, Duisters RF, Schellings MW, Janssen BJ, Debets JJ, Schwake M, Høydal MA, Heymans S, Saftig P, Pinto YM.
J Exp Med. 2007 May 14;204(5):1227-35

105

Self-assembly of the isolated KCNQ2 subunit interaction domain.
Wehling C, Beimgraben C, Gelhaus C, Friedrich T, Saftig P, Grötzinger J, Schwake M.
FEBS Lett. 2007 Apr 17;581(8):1594-8

104

Cathepsin D deficiency induces persistent neurodegeneration in the absence of Bax-dependent apoptosis.
Shacka JJ, Klocke BJ, Young C, Shibata M, Olney JW, Uchiyama Y, Saftig P, Roth KA.
J Neurosci. 2007 Feb 21;27(8):2081-90

103

Metalloproteases regulate T-cell proliferation and effector function via LAG-3.
Li N, Wang Y, Forbes K, Vignali KM, Heale BS, Saftig P, Hartmann D, Black RA, Rossi JJ, Blobel CP, Dempsey PJ, Workman CJ, Vignali DA.
EMBO J. 2007 Jan 24;26(2):494-504

102

LAMP proteins are required for fusion of lysosomes with phagosomes.
Huynh KK, Eskelinen EL, Scott CC, Malevanets A, Saftig P, Grinstein S.
EMBO J. 2007 Jan 24;26(2):313-24

101

ADAM10 inhibition of human CD30 shedding increases specificity of targeted immunotherapy in vitro.
Eichenauer DA, Simhadri VL, von Strandmann EP, Ludwig A, Matthews V, Reiners KS, von Tresckow B, Saftig P, Rose-John S, Engert A, Hansen HP.
Cancer Res. 2007 Jan 1;67(1):332-8

100

Substrate selectivity of epidermal growth factor-receptor ligand sheddases and their regulation by phorbol esters and calcium influx.
Horiuchi K, Le Gall S, Schulte M, Yamaguchi T, Reiss K, Murphy G, Toyama Y, Hartmann D, Saftig P, Blobel CP.
Mol Biol Cell. 2007 Jan;18(1):176-88. Erratum in: Mol Biol Cell. 2010 Jun;21(11):1908.

99

ADAM10 is a principal 'sheddase' of the low-affinity immunoglobulin E receptor CD23.
Weskamp G, Ford JW, Sturgill J, Martin S, Docherty AJ, Swendeman S, Broadway N, Hartmann D, Saftig P, Umland S, Sehara-Fujisawa A, Black RA, Ludwig A, Becherer JD, Conrad DH, Blobel CP.
Nat Immunol. 2006 Dec;7(12):1293-8

98

Cathepsin protease activity modulates amyloid load in extracerebral amyloidosis.
Röcken C, Fändrich M, Stix B, Tannert A, Hortschansky P, Reinheckel T, Saftig P, Kähne T, Menard R, Ancsin JB, Bühling F.
J Pathol. 2006 Dec;210(4):478-87

97

Gene profiling of cathepsin K deficiency in atherogenesis: profibrotic but lipogenic.
Lutgens SP, Kisters N, Lutgens E, van Haaften RI, Evelo CT, de Winther MP, Saftig P, Daemen MJ, Heeneman S, Cleutjens KB.
J Pathol. 2006 Nov;210(3):334-43

96

Calvarial osteoclasts express a higher level of tartrate-resistant acid phosphatase than long bone osteoclasts and activation does not depend on cathepsin K or L activity.
Perez-Amodio S, Jansen DC, Schoenmaker T, Vogels IM, Reinheckel T, Hayman AR, Cox TM, Saftig P, Beertsen W, Everts V.
Calcif Tissue Int. 2006 Oct;79(4):245-54

95

Control of peripheral nerve myelination by the beta-secretase BACE1.
Willem M, Garratt AN, Novak B, Citron M, Kaufmann S, Rittger A, DeStrooper B, Saftig P, Birchmeier C, Haass C.
Science. 2006 Oct 27;314(5799):664-6

94

Deafness in LIMP2-deficient mice due to early loss of the potassium channel KCNQ1/KCNE1 in marginal cells of the stria vascularis.
Knipper M, Claussen C, Rüttiger L, Zimmermann U, Lüllmann-Rauch R, Eskelinen EL, Schröder J, Schwake M, Saftig P.
J Physiol. 2006 Oct 1;576(Pt 1):73-86

93

Osteoclastic bone degradation and the role of different cysteine proteinases and matrix metalloproteinases: differences between calvaria and long bone.
Everts V, Korper W, Hoeben KA, Jansen ID, Bromme D, Cleutjens KB, Heeneman S, Peters C, Reinheckel T, Saftig P, Beertsen W.
J Bone Miner Res. 2006 Sep;21(9):1399-408

92

Regulated ADAM10-dependent ectodomain shedding of gamma-protocadherin C3 modulates cell-cell adhesion.
Reiss K, Maretzky T, Haas IG, Schulte M, Ludwig A, Frank M, Saftig P.
J Biol Chem. 2006 Aug 4;281(31):21735-44

91

Multivariate neurocognitive and emotional profile of a mannosidosis murine model for therapy assessment.
Caeyenberghs K, Balschun D, Roces DP, Schwake M, Saftig P, D’Hooge R.
Neurobiol Dis. 2006 Aug;23(2):422-32

90

Proteomic identification of desmoglein 2 and activated leukocyte cell adhesion molecule as substrates of ADAM17 and ADAM10 by difference gel electrophoresis.Bech-Serra JJ, Santiago-Josefat B, Esselens C, Saftig P, Baselga J, Arribas J, Canals F.
Mol Cell Biol. 2006 Jul;26(13):5086-95

89

LAMP-2 deficient mice show depressed cardiac contractile function without significant changes in calcium handling.
Stypmann J, Janssen PM, Prestle J, Engelen MA, Kögler H, Lüllmann-Rauch R, Eckardt L, von Figura K, Landgrebe J, Mleczko A, Saftig P.
Basic Res Cardiol. 2006 Jul;101(4):281-91

88

Cathepsin D deficiency is associated with a human neurodegenerative disorder.
Steinfeld R, Reinhardt K, Schreiber K, Hillebrand M, Kraetzner R, Bruck W, Saftig P, Gartner J.
Am J Hum Genet. 2006 Jun;78(6):988-98

87

Furin-, ADAM 10-, and gamma-secretase-mediated cleavage of a receptor tyrosine phosphatase and regulation of beta-catenin's transcriptional activity.
Anders L, Mertins P, Lammich S, Murgia M, Hartmann D, Saftig P, Haass C, Ullrich A.
Mol Cell Biol. 2006 May;26(10):3917-34

86

Structural determinants of M-type KCNQ (Kv7) K+ channel assembly.
Schwake M, Athanasiadu D, Beimgraben C, Blanz J, Beck C, Jentsch TJ, Saftig P, Friedrich T.
J Neurosci. 2006 Apr 5;26(14):3757-66

85

Cutting edge: a novel nonoxidative phagosomal mechanism exerted by cathepsin-D controls Listeria monocytogenes intracellular growth.
del Cerro-Vadillo E*, Madrazo-Toca F*, Carrasco-Marín E, Fernandez-Prieto L, Beck C, Leyva-Cobián F, Saftig P*, Alvarez-Dominguez C*.
J Immunol. 2006 Feb 1;176(3):1321-5
(* equally contributed)

84

Disruption of the cathepsin K gene reduces atherosclerosis progression and induces plaque fibrosis but accelerates macrophage foam cell formation.
Lutgens E, Lutgens SP, Faber BC, Heeneman S, Gijbels MM, de Winther MP, Frederik P, van der Made I, Daugherty A, Sijbers AM, Fisher A, Long CJ, Saftig P, Black D, Daemen MJ, Cleutjens KB.
Circulation. 2006 Jan 3;113(1):98-107

83

Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation.Willenborg M, Schmidt CK, Braun P, Landgrebe J, von Figura K, Saftig P*, Eskelinen EL*.
J Lipid Res. 2005 Dec;46(12):2559-69
(* contributed equally)

82

Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease).
Koike M, Shibata M, Waguri S, Yoshimura K, Tanida I, Kominami E, Gotow T, Peters C, von Figura K, Mizushima N, Saftig P, Uchiyama Y.
Am J Pathol. 2005 Dec;167(6):1713-28

81

Unifying nomenclature for the isoforms of the lysosomal membrane protein LAMP-2.
Eskelinen EL, Cuervo AM, Taylor MR, Nishino I, Blum JS, Dice JF, Sandoval IV, Lippincott-Schwartz J, August JT, Saftig P.
Traffic. 2005 Nov;6(11):1058-61

80

L1 is sequentially processed by two differently activated metalloproteases and presenilin/gamma-secretase and regulates neural cell adhesion, cell migration, and neurite outgrowth.
Maretzky T, Schulte M, Ludwig A, Rose-John S, Blobel C, Hartmann D, Altevogt P, Saftig P, Reiss K.
Mol Cell Biol. 2005 Oct;25(20):9040-53

79

The NH(2)-terminal transmembrane and lumenal domains of LGP85 are needed for the formation of enlarged endosomes/lysosomes.
Kuronita T, Hatano T, Furuyama A, Hirota Y, Masuyama N, Saftig P, Himeno M, Fujita H, Tanaka Y.
Traffic. 2005 Oct;6(10):895-906

78

Phenotypic and biochemical analyses of BACE1- and BACE2-deficient mice.
Dominguez D, Tournoy J, Hartmann D, Huth T, Cryns K, Deforce S, Serneels L, Camacho IE, Marjaux E, Craessaerts K, Roebroek AJ, Schwake M, D’Hooge R, Bach P, Kalinke U, Moechars D, Alzheimer C, Reiss K, Saftig P, De Strooper B.
J Biol Chem. 2005 Sep 2;280(35):30797-806

77

Proteolytic degradation of glutamate decarboxylase mediates disinhibition of hippocampal CA3 pyramidal cells in cathepsin D-deficient mice.Shimizu T, Hayashi Y, Yamasaki R, Yamada J, Zhang J, Ukai K, Koike M, Mine K, von Figura K, Peters C, Saftig P, Fukuda T, Uchiyama Y, Nakanishi H.
J Neurochem. 2005 Aug;94(3):680-90

76

Neurocognitive and psychotiform behavioral alterations and enhanced hippocampal long-term potentiation in transgenic mice displaying neuropathological features of human alpha-mannosidosis.
D’Hooge R, Lüllmann-Rauch R, Beckers T, Balschun D, Schwake M, Reiss K, von Figura K, Saftig P.
J Neurosci. 2005 Jul 13;25(28):6539-49

75

The apoptosis/autophagy paradox: autophagic vacuolization before apoptotic death.
González-Polo RA, Boya P, Pauleau AL, Jalil A, Larochette N, Souquère S, Eskelinen EL, Pierron G, Saftig P, Kroemer G.
J Cell Sci. 2005 Jul 15;118(Pt 14):3091-102

74

ADAM10 mediates E-cadherin shedding and regulates epithelial cell-cell adhesion, migration, and beta-catenin translocation.
Maretzky T, Reiss K, Ludwig A, Buchholz J, Scholz F, Proksch E, de Strooper B, Hartmann D, Saftig P.
Proc Natl Acad Sci U S A. 2005 Jun 28;102(26):9182-7

73

beta Subunits of voltage-gated sodium channels are novel substrates of beta-site amyloid precursor protein-cleaving enzyme (BACE1) and gamma-secretase.
Wong HK, Sakurai T, Oyama F, Kaneko K, Wada K, Miyazaki H, Kurosawa M, De Strooper B, Saftig P, Nukina N.
J Biol Chem. 2005 Jun 17;280(24):23009-17

72

Autophagic vacuoles with sarcolemmal features delineate Danon disease and related myopathies.
Sugie K, Noguchi S, Kozuka Y, Arikawa-Hirasawa E, Tanaka M, Yan C, Saftig P, von Figura K, Hirano M, Ueno S, Nonaka I, Nishino I.
J Neuropathol Exp Neurol. 2005 Jun;64(6):513-22

71

Molecular determinants of KCNQ (Kv7) K+ channel sensitivity to the anticonvulsant retigabine.
Schenzer A, Friedrich T, Pusch M, Saftig P, Jentsch TJ, Grötzinger J, Schwake M.
J Neurosci. 2005 May 18;25(20):5051-60

70

A role for cathepsin E in the processing of mast-cell carboxypeptidase A.
Henningsson F, Yamamoto K, Saftig P, Reinheckel T, Peters C, Knight SD, Pejler G.
J Cell Sci. 2005 May 1;118(Pt 9):2035-42

69

ADAM10 cleavage of N-cadherin and regulation of cell-cell adhesion and beta-catenin nuclear signalling.
Reiss K, Maretzky T, Ludwig A, Tousseyn T, de Strooper B, Hartmann D, Saftig P.
EMBO J. 2005 Feb 23;24(4):742-52. Erratum in: EMBO J. 2005 May 4;24(9):1762.

68

Dimerization of beta-site beta-amyloid precursor protein-cleaving enzyme.
Westmeyer GG, Willem M, Lichtenthaler SF, Lurman G, Multhaup G, Assfalg-Machleidt I, Reiss K, Saftig P, Haass C.
J Biol Chem. 2004 Dec 17;279(51):53205-12

67

Altered morphological and electrophysiological properties of Cajal-Retzius cells in cerebral cortex of embryonic Presenilin-1 knockout mice.
Kilb W, Hartmann D, Saftig P, Luhmann HJ.
Eur J Neurosci. 2004 Nov;20(10):2749-56

66

Cathepsin K: a cysteine protease with unique kinin-degrading properties.
Godat E, Lecaille F, Desmazes C, Duchêne S, Weidauer E, Saftig P, Brömme D, Vandier C, Lalmanach G.
Biochem J. 2004 Nov 1;383(Pt. 3):501-6

65

Evaluation of the contribution of different ADAMs to tumor necrosis factor alpha (TNFalpha) shedding and of the function of the TNFalpha ectodomain in ensuring selective stimulated shedding by the TNFalpha convertase (TACE/ADAM17).
Zheng Y, Saftig P, Hartmann D, Blobel C.
J Biol Chem. 2004 Oct 8;279(41):42898-906

64

Presenilin 1 mediates the turnover of telencephalin in hippocampal neurons via an autophagic degradative pathway.
Esselens C, Oorschot V, Baert V, Raemaekers T, Spittaels K, Serneels L, Zheng H, Saftig P, De Strooper B, Klumperman J, Annaert W.
J Cell Biol. 2004 Sep 27;166(7):1041-54

63

Efficacy of enzyme replacement therapy in alpha-mannosidosis mice: a preclinical animal study.
Roces DP, Lüllmann-Rauch R, Peng J, Balducci C, Andersson C, Tollersrud O, Fogh J, Orlacchio A, Beccari T, Saftig P, von Figura K.
Hum Mol Genet. 2004 Sep 15;13(18):1979-88

62

Role for Rab7 in maturation of late autophagic vacuoles.
Jäger S, Bucci C, Tanida I, Ueno T, Kominami E, Saftig P, Eskelinen EL.
J Cell Sci. 2004 Sep 15;117(Pt 20):4837-48

61

Cathepsin L is involved in cathepsin D processing and regulation of apoptosis in A549 human lung epithelial cells.
Wille A, Gerber A, Heimburg A, Reisenauer A, Peters C, Saftig P, Reinheckel T, Welte T, Bühling F.
Biol Chem. 2004 Jul;385(7):665-70

60

Partial loss of presenilins causes seborrheic keratosis and autoimmune disease in mice.
Tournoy J, Bossuyt X, Snellinx A, Regent M, Garmyn M, Serneels L, Saftig P, Craessaerts K, De Strooper B, Hartmann D.
Hum Mol Genet. 2004 Jul 1;13(13):1321-31

59

Disturbed cholesterol traffic but normal proteolytic function in LAMP-1/LAMP-2 double-deficient fibroblasts.Eskelinen EL, Schmidt CK, Neu S, Willenborg M, Fuertes G, Salvador N, Tanaka Y, Lüllmann-Rauch R, Hartmann D, Heeren J, von Figura K, Knecht E, Saftig P.
Mol Biol Cell. 2004 Jul;15(7):3132-45

58

Cell-matrix interaction via CD44 is independently regulated by different metalloproteinases activated in response to extracellular Ca(2+) influx and PKC activation.
Nagano O, Murakami D, Hartmann D, De Strooper B, Saftig P, Iwatsubo T, Nakajima M, Shinohara M, Saya H.
J Cell Biol. 2004 Jun 21;165(6):893-902

57

Pivotal role of cathepsin K in lung fibrosis.
Bühling F, Röcken C, Brasch F, Hartig R, Yasuda Y, Saftig P, Brömme D, Welte T.
Am J Pathol. 2004 Jun;164(6):2203-16

56

Cathepsin D is involved in the regulation of transglutaminase 1 and epidermal differentiation.
Egberts F, Heinrich M, Jensen JM, Winoto-Morbach S, Pfeiffer S, Wickel M, Schunck M, Steude J, Saftig P, Proksch E, Schütze S.
J Cell Sci. 2004 May 1;117(Pt 11):2295-307

55

The transmembrane CXC-chemokine ligand 16 is induced by IFN-gamma and TNF-alpha and shed by the activity of the disintegrin-like metalloproteinase ADAM10.
Abel S, Hundhausen C, Mentlein R, Schulte A, Berkhout TA, Broadway N, Hartmann D, Sedlacek R, Dietrich S, Muetze B, Schuster B, Kallen KJ, Saftig P, Rose-John S, Ludwig A.
J Immunol. 2004 May 15;172(10):6362-72

54

Cathepsin D is involved in the regulation of transglutaminase 1 and epidermal differentiation.
Egberts F, Heinrich M, Jensen JM, Winoto-Morbach S, Pfeiffer S, Wickel M, Schunck M, Steude J, Saftig P, Proksch E, Schütze S.
J Cell Sci. 2004 May 1;117(Pt 11):2295-307

53

Cathepsin D is up-regulated in inflammatory bowel disease macrophages.
Hausmann M, Obermeier F, Schreiter K, Spottl T, Falk W, Schölmerich J, Herfarth H, Saftig P, Rogler G.
Clin Exp Immunol. 2004 Apr;136(1):157-67

52

Distinct roles for ADAM10 and ADAM17 in ectodomain shedding of six EGFR ligands.
Sahin U, Weskamp G, Kelly K, Zhou HM, Higashiyama S, Peschon J, Hartmann D, Saftig P, Blobel CP.
J Cell Biol. 2004 Mar 1;164(5):769-79.

51

Evidence for a critical role of the tumor necrosis factor alpha convertase (TACE) in ectodomain shedding of the p75 neurotrophin receptor (p75NTR).
Weskamp G, Schlöndorff J, Lum L, Becherer JD, Kim TW, Saftig P, Hartmann D, Murphy G, Blobel CP.
J Biol Chem. 2004 Feb 6;279(6):4241-9

50

Deficiency of mannose 6-phosphate receptors and lysosomal storage: a morphometric analysis of hepatocytes of neonatal mice.
Schellens JP, Saftig P, von Figura K, Everts V.
Cell Biol Int. 2003;27(11):897-902

49

The cell adhesion protein P-selectin glycoprotein ligand-1 is a substrate for the aspartyl protease BACE1.
Lichtenthaler SF, Dominguez DI, Westmeyer GG, Reiss K, Haass C, Saftig P, De Strooper B, Seed B.
J Biol Chem. 2003 Dec 5;278(49):48713-9.

48

Cellular cholesterol depletion triggers shedding of the human interleukin-6 receptor by ADAM10 and ADAM17 (TACE).
Matthews V, Schuster B, Schütze S, Bussmeyer I, Ludwig A, Hundhausen C, Sadowski T, Saftig P, Hartmann D, Kallen KJ, Rose-John S.
J Biol Chem. 2003 Oct 3;278(40):38829-39

47

Cathepsin K deficiency in pycnodysostosis results in accumulation of non-digested phagocytosed collagen in fibroblasts.
Everts V, Hou WS, Rialland X, Tigchelaar W, Saftig P, Brömme D, Gelb BD, Beertsen W.
Calcif Tissue Int. 2003 Oct;73(4):380-6

46

The disintegrin-like metalloproteinase ADAM10 is involved in constitutive cleavage of CX3CL1 (fractalkine) and regulates CX3CL1-mediated cell-cell adhesion.
Hundhausen C, Misztela D, Berkhout TA, Broadway N, Saftig P, Reiss K, Hartmann D, Fahrenholz F, Postina R, Matthews V, Kallen KJ, Rose-John S, Ludwig A.
Blood. 2003 Aug 15;102(4):1186-95

45

Thyroid functions of mouse cathepsins B, K, and L.
Friedrichs B, Tepel C, Reinheckel T, Deussing J, von Figura K, Herzog V, Peters C, Saftig P, Brix K.
J Clin Invest. 2003 Jun;111(11):1733-45

44

LIMP-2/LGP85 deficiency causes ureteric pelvic junction obstruction, deafness and peripheral neuropathy in mice.
Gamp AC, Tanaka Y, Lüllmann-Rauch R, Wittke D, D’Hooge R, De Deyn PP, Moser T, Maier H, Hartmann D, Reiss K, Illert AL, von Figura K, Saftig P.
Hum Mol Genet. 2003 Mar 15;12(6):631-46

43

Involvement of two different cell death pathways in retinal atrophy of cathepsin D-deficient mice.
Koike M, Shibata M, Ohsawa Y, Nakanishi H, Koga T, Kametaka S, Waguri S, Momoi T, Kominami E, Peters C, Figura Kv, Saftig P, Uchiyama Y.
Mol Cell Neurosci. 2003 Feb;22(2):146-61

42

A role for the lysosomal membrane protein LGP85 in the biogenesis and maintenance of endosomal and lysosomal morphology.
Kuronita T, Eskelinen EL, Fujita H, Saftig P, Himeno M, Tanaka Y.
J Cell Sci. 2002 Nov 1;115(Pt 21):4117-31

41

The disintegrin/metalloprotease ADAM 10 is essential for Notch signalling but not for alpha-secretase activity in fibroblasts.
Hartmann D, de Strooper B, Serneels L, Craessaerts K, Herreman A, Annaert W, Umans L, Lübke T, Lena Illert A, von Figura K, Saftig P.
Hum Mol Genet. 2002 Oct 1;11(21):2615-24

40

Role of LAMP-2 in lysosome biogenesis and autophagy.
Eskelinen EL, Illert AL, Tanaka Y, Schwarzmann G, Blanz J, Von Figura K, Saftig P.
Mol Biol Cell. 2002 Sep;13(9):3355-68

39

No endogenous A beta production in presenilin-deficient fibroblasts.
Nyabi O, Pype S, Mercken M, Herreman A, Saftig P, Craessaerts K, Serneels L, Annaert W, De Strooper B.
Nat Cell Biol. 2002 Jul;4(7):E164; author reply E165-6

38

A physiologic signaling role for the gamma-secretase-derived intracellular fragment of APP.
Leissring MA, Murphy MP, Mead TR, Akbari Y, Sugarman MC, Jannatipour M, Anliker B, Müller U, Saftig P, De Strooper B, Wolfe MS, Golde TE, LaFerla FM.
Proc Natl Acad Sci U S A. 2002 Apr 2;99(7):4697-702

37

Reduced sperm count and normal fertility in male mice with targeted disruption of the ADP-ribosylation factor-like 4 (Arl4) gene.
Schürmann A, Koling S, Jacobs S, Saftig P, Krauss S, Wennemuth G, Kluge R, Joost HG.
Mol Cell Biol. 2002 Apr;22(8):2761-8.

36

Involvement of cathepsin E in exogenous antigen processing in primary cultured murine microglia.
Nishioku T, Hashimoto K, Yamashita K, Liou SY, Kagamiishi Y, Maegawa H, Katsube N, Peters C, von Figura K, Saftig P, Katunuma N, Yamamoto K, Nakanishi H.
J Biol Chem. 2002 Feb 15;277(7):4816-22

35

Decreased intracellular degradation of insulin-like growth factor binding protein-3 in cathepsin L-deficient fibroblasts.Zwad O, Kübler B, Roth W, Scharf JG, Saftig P, Peters C, Braulke T.
FEBS Lett. 2002 Jan 16;510(3):211-5

34

The bone lining cell: its role in cleaning Howship's lacunae and initiating bone formation.
Everts V, Delaissé JM, Korper W, Jansen DC, Tigchelaar-Gutter W, Saftig P, Beertsen W.
J Bone Miner Res. 2002 Jan;17(1):77-90

33

Overlapping functions of lysosomal acid phosphatase (LAP) and tartrate-resistant acid phosphatase (Acp5) revealed by doubly deficient mice.Suter A, Everts V, Boyde A, Jones SJ, Lüllmann-Rauch R, Hartmann D, Hayman AR, Cox TM, Evans MJ, Meister T, von Figura K, Saftig P.
Development. 2001 Dec;128(23):4899-910

32

Elevation of beta-amyloid peptide 2-42 in sporadic and familial Alzheimer's disease and its generation in PS1 knockout cells.
Wiltfang J, Esselmann H, Cupers P, Neumann M, Kretzschmar H, Beyermann M, Schleuder D, Jahn H, Rüther E, Kornhuber J, Annaert W, De Strooper B, Saftig P.
J Biol Chem. 2001 Nov 16;276(46):42645-57

31

The disintegrins ADAM10 and TACE contribute to the constitutive and phorbol ester-regulated normal cleavage of the cellular prion protein.
Vincent B, Paitel E, Saftig P, Frobert Y, Hartmann D, De Strooper B, Grassi J, Lopez-Perez E, Checler F.
J Biol Chem. 2001 Oct 12;276(41):37743-6

30

Involvement of nitric oxide released from microglia-macrophages in pathological changes of cathepsin D-deficient mice.Nakanishi H, Zhang J, Koike M, Nishioku T, Okamoto Y, Kominami E, von Figura K, Peters C, Yamamoto K, Saftig P, Uchiyama Y.
J Neurosci. 2001 Oct 1;21(19):7526-33

29

The discrepancy between presenilin subcellular localization and gamma-secretase processing of amyloid precursor protein.
Cupers P, Bentahir M, Craessaerts K, Orlans I, Vanderstichele H, Saftig P, De Strooper B, Annaert W.
J Cell Biol. 2001 Aug 20;154(4):731-40

28

Biosynthesis of phosphatidylcholine from a phosphocholine precursor pool derived from the late endosomal/lysosomal degradation of sphingomyelin.
Jansen SM, Groener JE, Bax W, Suter A, Saftig P, Somerharju P, Poorthuis BJ.
J Biol Chem. 2001 Jun 1;276(22):18722-7

27

Cathepsin L deficiency as molecular defect of furless: hyperproliferation of keratinocytes and pertubation of hair follicle cycling.
Roth W, Deussing J, Botchkarev VA, Pauly-Evers M, Saftig P, Hafner A, Schmidt P, Schmahl W, Scherer J, Anton-Lamprecht I, Von Figura K, Paus R, Peters C.
FASEB J. 2000 Oct;14(13):2075-86

26

Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons.
Koike M, Nakanishi H, Saftig P, Ezaki J, Isahara K, Ohsawa Y, Schulz-Schaeffer W, Watanabe T, Waguri S, Kametaka S, Shibata M, Yamamoto K, Kominami E, Peters C, von Figura K, Uchiyama Y.
J Neurosci. 2000 Sep 15;20(18):6898-906

25

Accumulation of autophagic vacuoles and cardiomyopathy in LAMP-2-deficient mice.
Tanaka Y, Guhde G, Suter A, Eskelinen EL, Hartmann D, Lüllmann-Rauch R, Janssen PM, Blanz J, von Figura K, Saftig P.
Nature. 2000 Aug 24;406(6798):902-6

24

Spermatogenesis proceeds normally in mice without linker histone H1t.
Drabent B, Saftig P, Bode C, Doenecke D.
Histochem Cell Biol. 2000 Jun;113(6):433-42

23

mu1A-adaptin-deficient mice: lethality, loss of AP-1 binding and rerouting of mannose 6-phosphate receptors.
Meyer C, Zizioli D, Lausmann S, Eskelinen EL, Hamann J, Saftig P, von Figura K, Schu P.
EMBO J. 2000 May 15;19(10):2193-203

22

Spermatogenesis in mice is not affected by histone H1.1 deficiency.
Rabini S, Franke K, Saftig P, Bode C, Doenecke D, Drabent B.
Exp Cell Res. 2000 Feb 25;255(1):114-24

21

Cell surface presenilin-1 participates in the gamma-secretase-like proteolysis of Notch.
Ray WJ, Yao M, Mumm J, Schroeter EH, Saftig P, Wolfe M, Selkoe DJ, Kopan R, Goate AM.
J Biol Chem. 1999 Dec 17;274(51):36801-7

20

Cathepsin D targeted by acid sphingomyelinase-derived ceramide.
Heinrich M, Wickel M, Schneider-Brachert W, Sandberg C, Gahr J, Schwandner R, Weber T, Saftig P, Peters C, Brunner J, Krönke M, Schütze S.
EMBO J. 1999 Oct 1;18(19):5252-63. Erratum in: EMBO J 2000 Jan 17;19(2):315.

19

Presenilin 2 deficiency causes a mild pulmonary phenotype and no changes in amyloid precursor protein processing but enhances the embryonic lethal phenotype of presenilin 1 deficiency.
Herreman A, Hartmann D, Annaert W, Saftig P, Craessaerts K, Serneels L, Umans L, Schrijvers V, Checler F, Vanderstichele H, Baekelandt V, Dressel R, Cupers P, Huylebroeck D, Zwijsen A, Van Leuven F, De Strooper B.
Proc Natl Acad Sci U S A. 1999 Oct 12;96(21):11872-7

18

Targeted disruption of the lysosomal alpha-mannosidase gene results in mice resembling a mild form of human alpha-mannosidosis.
Stinchi S, Lüllmann-Rauch R, Hartmann D, Coenen R, Beccari T, Orlacchio A, von Figura K, Saftig P.
Hum Mol Genet. 1999 Aug;8(8):1365-72

17

Presenilin-1 deficiency leads to loss of Cajal-Retzius neurons and cortical dysplasia similar to human type 2 lissencephaly.
Hartmann D, De Strooper B, Saftig P.
Curr Biol. 1999 Jul 15;9(14):719-27

16

Normal lysosomal morphology and function in LAMP-1-deficient mice.
Andrejewski N, Punnonen EL, Guhde G, Tanaka Y, Lüllmann-Rauch R, Hartmann D, von Figura K, Saftig P.
J Biol Chem. 1999 Apr 30;274(18):12692-701

15

A presenilin-1-dependent gamma-secretase-like protease mediates release of Notch intracellular domain.
De Strooper B, Annaert W, Cupers P, Saftig P, Craessaerts K, Mumm JS, Schroeter EH, Schrijvers V, Wolfe MS, Ray WJ, Goate A, Kopan R.
Nature. 1999 Apr 8;398(6727):518-22

14

Early embryonic death of mice deficient in gamma-adaptin.
Zizioli D, Meyer C, Guhde G, Saftig P, von Figura K, Schu P.
J Biol Chem. 1999 Feb 26;274(9):5385-90

13

Impaired osteoclastic bone resorption leads to osteopetrosis in cathepsin-K-deficient mice.
Saftig P, Hunziker E, Wehmeyer O, Jones S, Boyde A, Rommerskirch W, Moritz JD, Schu P, von Figura K.
Proc Natl Acad Sci U S A. 1998 Nov 10;95(23):13453-8

12

Destabilization of beta-catenin by mutations in presenilin-1 potentiates neuronal apoptosis.
Zhang Z, Hartmann H, Do VM, Abramowski D, Sturchler-Pierrat C, Staufenbiel M, Sommer B, van de Wetering M, Clevers H, Saftig P, De Strooper B, He X, Yankner BA.
Nature. 1998 Oct 15;395(6703):698-702

11

Potential role for cathepsin D in p53-dependent tumor suppression and chemosensitivity.
Wu GS, Saftig P, Peters C, El-Deiry WS.
Oncogene. 1998 Apr 30;16(17):2177-83

10

Cathepsins B and D are dispensable for major histocompatibility complex class II-mediated antigen presentation.
Deussing J, Roth W, Saftig P, Peters C, Ploegh HL, Villadangos JA.
Proc Natl Acad Sci U S A. 1998 Apr 14;95(8):4516-21

9

Deficiency of presenilin-1 inhibits the normal cleavage of amyloid precursor protein.
De Strooper B, Saftig P, Craessaerts K, Vanderstichele H, Guhde G, Annaert W, Von Figura K, Van Leuven F.
Nature. 1998 Jan 22;391(6665):387-90

8

Non-conventional role of lysosomal acid phosphatase in olfactory receptor axons: co-localization with growth-associated phosphoprotein-43.
Wolff JR, Liu WL, Böttcher H, Krizbai I, Jóo F, Saftig P, Parducz Á.
Neuroscience. 1997 Aug;79(3):887-91

7

Mice deficient in lysosomal acid phosphatase develop lysosomal storage in the kidney and central nervous system.
Saftig P, Hartmann D, Lüllmann-Rauch R, Wolff J, Evers M, Köster A, Hetman M, von Figura K, Peters C.
J Biol Chem. 1997 Jul 25;272(30):18628-35

6

Phenotype of arylsulfatase A-deficient mice: relationship to human metachromatic leukodystrophy.
Hess B, Saftig P, Hartmann D, Coenen R, Lüllmann-Rauch R, Goebel HH, Evers M, von Figura K, D’Hooge R, Nagels G, De Deyn P, Peters C, Gieselmann V.
Proc Natl Acad Sci U S A. 1996 Dec 10;93(25):14821-6

5

Amyloidogenic processing of human amyloid precursor protein in hippocampal neurons devoid of cathepsin D.
Saftig P, Peters C, von Figura K, Craessaerts K, Van Leuven F, De Strooper B.
J Biol Chem. 1996 Nov 1;271(44):27241-4

4

Targeted disruption of the arylsulfatase B gene results in mice resembling the phenotype of mucopolysaccharidosis VI.
Evers M, Saftig P, Schmidt P, Hafner A, McLoghlin DB, Schmahl W, Hess B, von Figura K, Peters C.
Proc Natl Acad Sci U S A. 1996 Aug 6;93(16):8214-9

3

Mice deficient for the lysosomal proteinase cathepsin D exhibit progressive atrophy of the intestinal mucosa and profound destruction of lymphoid cells.
Saftig P, Hetman M, Schmahl W, Weber K, Heine L, Mossmann H, Köster A, Hess B, Evers M, von Figura K, et al.
EMBO J. 1995 Aug 1;14(15):3599-608

2

Mouse cathepsin D gene: molecular organization, characterization of the promoter, and chromosomal localization.
Hetman M, Perschl A, Saftig P, Von Figura K, Peters C.
DNA Cell Biol. 1994 Apr;13(4):419-27

1

Targeted disruption of the M(r) 46,000 mannose 6-phosphate receptor gene in mice results in misrouting of lysosomal proteins.
Köster A, Saftig P, Matzner U, von Figura K, Peters C, Pohlmann R.
EMBO J. 1993 Dec 15;12(13):5219-23

42

New insights into the function and pathophysiology of the ectodomain sheddase A Disintegrin And Metalloproteinase 10 (ADAM10).
Rosenbaum D, Saftig P.
FEBS J, in press.

41

Ectodomain shedding by ADAM proteases as a central regulator in kidney physiology and disease.
Meyer-Schwesinger, C., Seipold, L., Saftig, P.
Biochim Biophys Acta Mol Cell Res. 2021 Oct 23;119165

40

How Lysosomes Sense, Integrate, and Cope with Stress.
Saftig P & Puertelano R. (2020)
Trends Biochem Sci. 2021 Feb;46(2):97-112

39

Cholesterol Handling in Lysosomes and Beyond.
Meng Y, Heybrock S, Neculai D, Saftig P.
Trends Cell Biol. 2020 Jun;30(6):452-466

38

Lysosomal storage disorders - challenges, concepts and avenues for therapy: beyond rare diseases.
Marques ARA, Saftig P.
J Cell Sci. 2019 Jan 16;132(2):jcs221739

37

Intracellular compartments of pathogens: Highways to hell or stairways to heaven?
Haas A, Hensel M, Lührmann A, Rudel T, Saftig P, Schaible UE.
Int J Med Microbiol. 2018 Jan;308(1):1-2

36

Vacuolar ATPase in phago(lyso)some biology.
Kissing S, Saftig P, Haas A.
Int J Med Microbiol. 2018 Jan;308(1):58-67
*: authors contributed equally

35

Diverse functions of the prion protein - Does proteolytic processing hold the key?
Linsenmeier L, Altmeppen HC, Wetzel S, Mohammadi B, Saftig P, Glatzel M.
Biochim Biophys Acta Mol Cell Res. 2017 Nov;1864(11 Pt B):2128-2137

34

The metalloproteinase ADAM10: A useful therapeutic target?
Wetzel S, Seipold L, Saftig P.
Biochim Biophys Acta Mol Cell Res. 2017 Nov;1864(11 Pt B):2071-2081

33

The Emerging Role of Tetraspanins in the Proteolytic Processing of the Amyloid Precursor Protein.
Seipold L, Saftig P.
Front Mol Neurosci. 2016 Dec 21;9:149. Review. Erratum in: Front Mol Neurosci. 2017 Feb 10;10 :37.

32

Turn up the lysosome.
Saftig P, Haas A.
Nat Cell Biol. 2016 Sep 28;18(10):1025-7.

31

Intramembrane proteolysis within lysosomes.
Schröder B, Saftig P.
Ageing Res Rev. 2016 Dec;32:51-64

30

Parkinson's disease: acid-glucocerebrosidase activity and alpha-synuclein clearance.
Blanz, J. & Saftig, P.
J Neurochem. 2016 Oct;139 Suppl 1:198-215.

29

The alpha secretase ADAM10: A metalloprotease with multiple functions in the brain.
Saftig P, Lichtenthaler SF
Prog Neurobiol. 2015 Dec;135:1-20

28

Proteases at work: cues for understanding neural development and degeneration.
Saftig P, Bovolenta P
Front Mol Neurosci. 2015 May 5;8:13

27

Autophagy in neuronal cells: general principles and physiological and pathological functions.
Damme M, Suntio T, Saftig P, Eskelinen EL
Acta Neuropathol. 2015 Mar;129(3):337-62

26

ADAM10: alpha-secretase and regulator in neurobiology.
Prox, J., Saftig, P
Neuroforum 2014; 2/14, 212-219

25

Cancer: Killing from the inside.
Saftig P, Sandhoff K
Nature. 2013 Oct 17;502(7471):312-3

24

Lysosomal membrane proteins and their central role in physiology.
Schwake M, Schröder B, Saftig P
Traffic. 2013 Jul;14(7):739-48

23

Ectodomain shedding and ADAMs in development.
Weber S, Saftig P
Development. 2012 Oct;139(20):3693-709

22

The "A Disintegrin And Metalloproteases" ADAM10 and ADAM17: novel drug targets with therapeutic potential?
Saftig P, Reiss K
Eur J Cell Biol. 2011 Jun-Jul;90(6-7):527-35

21

Lysosomal membrane proteins: life between acid and neutral conditions.
Saftig P, Schröder B, Blanz J
Biochem Soc Trans. 2010 Dec;38(6):1420-3

20

The proteome of lysosomes.
Schröder BA, Wrocklage C, Hasilik A, Saftig P
Proteomics. 2010 Nov;10(22):4053-76

19

Lysosome biogenesis and lysosomal membrane proteins: trafficking meets function.
Saftig P, Klumperman J
Nat Rev Mol Cell Biol. 2009 Sep;10(9):623-35

18

The "a disintegrin and metalloprotease" (ADAM) family of sheddases: physiological and cellular functions.
Reiss K, Saftig P
Semin Cell Dev Biol. 2009 Apr;20(2):126-37

17

Annaert WG, Saftig P
Regulated intramembrane proteolysis--a story about sheddases and I-CliPs.
Semin Cell Dev Biol. 2009 Apr;20(2):125

16

Autophagy: a lysosomal degradation pathway with a central role in health and disease.
Eskelinen EL, Saftig P
Biochim Biophys Acta. 2009 Apr;1793(4):664-73

15

Lysosomal myopathies: an excessive build-up in autophagosomes is too much to handle.
Malicdan MC, Noguchi S, Nonaka I, Saftig P, Nishino I
Neuromuscul Disord. 2008 Jul;18(7):521-9

14

LAMP-2: a control step for phagosome and autophagosome maturation.
Saftig P, Beertsen W, Eskelinen EL
Autophagy. 2008 May;4(4):510-2

13

Live longer with LAMP-2.
Saftig P, Eskelinen EL
Nat Med. 2008 Sep;14(9):909-10

12

Breaking up the tie: disintegrin-like metalloproteinases as regulators of cell migration in inflammation and invasion.
Reiss K, Ludwig A, Saftig P
Pharmacol Ther. 2006 Sep;111(3):985-1006

11

At the acidic edge: emerging functions for lysosomal membrane proteins.
Eskelinen EL, Tanaka Y, Saftig P
Trends Cell Biol. 2003 Mar;13(3):137-45

10

Implication of APP secretases in notch signaling.
Hartmann D, Tournoy J, Saftig P, Annaert W, De Strooper BJ
Mol Neurosci. 2001 Oct;17(2):171-81

9

Funktionsanalyse lysosomaler Proteine mit Hilfe von Knockout-Mäusen
P. Saftig, K. von Figura
BIOspektrum 2001; 7, 528-532

8

Disease model: LAMP-2 enlightens Danon disease.
Saftig P, Tanaka Y, Lüllmann-Rauch R, von Figura K
Trends Mol Med 2001 Jan; 7(1):37-9.

7

Proteases involved in MHC class II antigen presentation.
Villadangos JA, Bryant RA, Deussing J, Driessen C, Lennon-Duménil AM, Riese RJ, Roth W, Saftig P, Shi GP, Chapman HA, Peters C, Ploegh HL
Immunol Rev 1999; 172:109-20.

6

The function of presenilin-1 in amyloid beta-peptide generation and brain development.
Saftig P, Hartmann D, De Strooper B
Eur Arch Psychiatry Clin Neurosci 1999; 249(6):271-9.

5

Function and dysfunction of the presenilins
Scheper W, Annaert W, Cupers P, Saftig P, De Strooper B
Alzheimer's Reports 1999 2, 73-81

4

The Two Major Familial Alzheimer's Disease Gene Products: Presenilin 1 and Amyloid Precursor Protein Interact Functionally.
Saftig P, Craessaerts K, Vanderstichele H, Guhde G, Annaert W, von Figura K, Van Leuven F, De Strooper B
NeuroScience News 1998 1, 35-38

3

Downregulation of PS1 expression in neurons decreases beta-amyloid production: a biochemical link between the two major familial Alzheimer's disease genes.
Saftig P, de Strooper B
Mol Psychiatry 1998 Jul;3(4):287-9

2

Deussing J, Pauly-Evers M, Roth W, Saftig P, Peters C
Manipulating the mouse genome.
Futura 1997; 12, 162-171

1

Proteolysis of IGFBPs by cathepsin D in vitro and in cathepsin D-deficient mice.
Braulke T, Claussen M, Saftig P, Wendland M, Neifer K, Schmidt B, Zapf J, von Figura K, Peters C
Prog Growth Factor Res 1995; 6(2-4):265-71

10

Schwake M, Saftig P (2022)
Lysosomal Membrane Defects. Chapter 21. In "Lysosomal Storage Disorders" A Practical Guide. Second Edition.
Ed. Atul B. Metha and Brian Winchester. Published 2022 by John Wiley and Sons Ltd.

9

Dibbens L, Schwake M, Saftig P, Rubboli G. (2016)
SCARB2/LIMP2 deficiency in action myoclonus-renal failure syndrome.
Epileptic Disord 18(S2):63-72.

8

Everts, V. & Saftig, P. (2011)
W.C. Parks and R.P. Mecham (eds), Extracellular Matrix Degradation, Biology of Extracellular matrix 2
DOI 10.1007/978-3-642-16861-1_8, Springer-Verlag Berlin Heidelberg 2011

7

P. Saftig (2005)
Lysosomal Membrane Proteins.
In: Lysosomes (ed. Paul Saftig), Springer, New York

6

P. Saftig and Dieter Hartmann (2005)
ADAM10: A major membrane protein ectodomain sheddase involved in regulated intramembrane proteolyis.
In: The ADAM Familiy of Proteases (ed. Nigel Hooper), Springer, New York

5

W. Annaert, P. Cupers, P. Saftig and B. De Strooper (2000)
Presenilin Function in APP Processing.
In: The Molecular Basis of Dementia (J.H. Growdon, R.J. Wurtman, S. Corkin, and R. M. Nitsch, Eds.) Annals New York Academy of Sciences, Vol. 920, pp. 158-164

4

M. Heinrich, M. Wickel, S. Winoto-Morbach, W. Schneider-Brachert, T. Weber, J. Brunner, P. Saftig, C. Peters, M. Krönke and S. Schütze (2000)
Ceramide as an Activator Lipid of Cathepsin D.
In: Cellular Peptidases in Immune Functions and Diseases 2, Adv. Exp. Med. Biol., vol. 477 (J. Langner and S. Ansorge, eds.) Kluwer Academic/Plenum Publishers, Amsterdam, pp. 304-316 (ISDBN 0-306-46383-0)

3

P. Saftig, E. Hunziker, V. Everts, S. Jones, A. Boyde, O. Wehmeyer, A. Suter, K. von Figura (2000)
Functions of Cathepsin K in Bone Resorption.
In: Cellular Peptidases in Immune Functions and Diseases 2, Adv. Exp. Med. Biol., vol. 477 (J. Langner and S. Ansorge, eds.) Kluwer Academic/Plenum Publishers, Amsterdam, pp. 293-303 (ISDBN 0-306-46383-0)

2

P. Cupers, W. Annaert, C. Boeve, K. Craessaerts, F. Van Leuven, P. Saftig, B. De Strooper (1999)
Modulation of the Proteolytic Processing of Amyloid Precursor Protein.
In: Recent Developments in Comparative Endocrinology and Neurobiology, ed. 1, pp. 58-60.
E.W. Roubos, S.E. Wendelaar Bonga, H. Vaudry, A. de Loof (Eds.)
Shaker Publishing, Maastricht, The Netherlands

1

P. Saftig, D. Hartmann, W. Annaert, K. Craessaerts, F. Van Leuven, B. De Strooper (1998)
The processing of the amyloid-precursor-protein (APP) in presenilin-1 deficient neurons.
In: Molecular Biology of Alzheimer's Disease (C. Haas, Ed.) Harwood Academic Publishers, Amsterdam, pp. 235-246

1

Lysosomes, Medical Intelligence Unit.
Landes Bioscience, Springer Science & Business Media, Inc. , ISBN: 0-387-25562-1; 2005
Editor: Paul Saftig