Collaborative Research Centre 877

Proteolysis as a Regulatory Event in Pathophysiology

Christian-Albrechts-Universität zu Kiel

Education and Academic Positions
2004–2007 Dissertation, Department of Biochemistry, Children’s Hospital, University Medical Center Hamburg-Eppendorf
2003–2004 Diploma Thesis, Biocenter Klein Flottbek, University of Hamburg
1998–2003 Studies of Biochemistry and Molecular Biology, University of Hamburg
Research Experience
Since 2016 Spokesperson of the Integrated Research Training Group "Proteolysis and Pathophysiology" (SFB 877)
Since 2007 Scientific Assistant and Principal Investigator at the Department of Biochemistry, Children’s Hospital, University Medical Center Hamburg-Eppendorf
2004–2007 PhD Student, Department of Biochemistry, Children’s Hospital, University Medical Center Hamburg-Eppendorf
Awards and Honors
2011 Award of the Werner Otto Foundation for Medical Research
Zeitungsausschnitt (BILD), Zeitungsausschnitt (Hamburger Abendblatt)
2011 Presentation Award, "dies academicus" of the Medical Faculty, Hamburg
2006 Young Investigator Award, Neuronal ceroid lipofuscinoses, EU-Consortium Meeting, Hamburg
List of 5 Selected Publications
1. Klünder S, Heeren J, Markmann S, Santer R, Braulke T, Pohl S (2015) Site-1 protease-activated formation of lysosomal targeting motifs is independent of the lipogenic transcription control. J Lipid Res 56: 1625-1632
2. Velho RV, De Pace R, Klünder S, Sperb-Ludwig F, Lourenço CM, Schwartz IV, Braulke T, Pohl S (2015) Analyses of disease-related GNPTAB mutations define a novel GlcNAc-1-phosphotransferase interaction domain and an alternative site-1 protease cleavage site. Hum Mol Genet 24: 3497-3505
3. De Pace R, Coutinho MF, Koch-Nolte F, Haag F, Prata MJ, Alves S, Braulke T, Pohl S (2014) Mucolipidosis II-related mutations inhibit the exit from the endoplasmic reticulum and proteolytic cleavage of GlcNAc-1-phosphotransferase precursor protein (GNPTAB). Hum Mutat 35: 368-376
4. Marschner K, Kollmann K, Schweizer M, Braulke T, Pohl S (2011) A key enzyme in the biogenesis of lysosomes is a protease that regulates cholesterol metabolism. Science 333: 87-90
5. Encarnação M, Kollmann K, Trusch M, Braulke T, Pohl S (2011) Post-translational modifications of the gamma-subunit affect intracellular trafficking and complex assembly of GlcNAc-1-phosphotransferase. J Biol Chem 286: 5311-5318