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Participant 1

Christian-Albrechts-University Kiel

Department of Biochemistry, University of Kiel, D-24018 Kiel, GERMANY (Team leader/Responsible Scientist: Prof. Dr. Paul Saftig Coordinator). Key skills: Enzyme replacement

Laboratory background and technical information: The department of Biochemistry is part of the medical faculty and engaged in teaching programs for students of medicine, biochemistry and biology. It has all facilities necessary for the mouse experiments and biochemical and cell biological analyses. The majority of projects is focussed on the biogenesis and function of lysosomes, lysosomal membrane proteins in mammalian cells. The institute has a long standing interest in genetic defects that result in lysosomal storage disorders. For a better understanding of the pathobiochemistry and treatment of lysosomal storage disorders of the functions of lysosomal proteins and of the components of their transport machinery mouse models are generated, in which one of the lysosomal proteins, e.g. phosphatases, proteases, lysosomal membrane proteins or transport factors is deficient. Mice deficient for the lysosomal hydrolases arylsulfatase A, arylsulfatase B, cathepsin-. and a-mannosidase were generated and serve as animal models for the human diseases mucopolysaccaridosis VI, metachromatic leucodystrophie (MLD), pynodystostosis and alpha-mannosidosis, respectively. Further research activities of the department are focussed around the function of lysosomal membrane proteins, presenilins and ADAM proteases.

Capacity to contribute to the project: Partner 1 has generated and characterised the a-mannosidase deficient mice. All the necessary equipment and facilities to perform experiments related to the enzyme replacement of a-mannosidase deficient mice is available. Animals are accomodated in conventional facilities in individually ventilated racks, injection in the tail vene of mice, biochemical analyses of neutral sugar contents, thin-layer chromatography of oligosacharaides, determination of enzyme activities are established methods in the laboratory of partner 1. Light- and electronmicrosocopy will be done in collaboration with Prof. Renate Lüllmann- Rauch, University of Kiel, Germany.

Members involved: To be supported by the project: One Postdoc who will be responsible for all of the described experiments involving enzyme replacement in a- mannosidase deficient mice. Apart from supervising the maintenance of the a- mannosidase deficient mouse colony and enzyme injections, the advanced biochemical and part of the morphological analyses will be performed by this person. (36 pm) Technical assistant: A technical assistant will be responsible for the processing of tissue specimen for light microscopy, immunocytochemistry, electronmicroscopy derived mock- and a-mannosidase injected mice. He/she will also be involved in the documentation of the morphological analysis. Furthermore the determination of mannose containing oligosaccharides in tissues of mock- and a- mannosidase injected mice will be performed using standard technique (neutral sugar determination, thin layer chromatography) (36 pm). One secretary who will assist in the management (WP12) of this consortium (18pm).

Other contributing members of the laboratory: Dr. Paul Saftig PhD., director of the institute, enzyme replacement therapy of a-mannosidase, monitoring lysosomal storage in a-mannosidase deficient mice. Supervision of enzyme replacement project (18 pm); Dr. Eeva Liisa Eskelinen, PhD, expert in electronmicroscopy and cell biology, advice (6 pm), Dr. Karina Reiss, PhD, expert in mouse experiments and analysis (12pm). Marlies Rusch, technical assistant: monitoring of the LAMAN preparations, Maintenance of mouse colony. (18 m); mrs Hohn, secretary: correspondence, manuscript processing, administration (6 pm). Mrs Holz, animal facility: daily animal care (6 pm).

Collaborators: prof. dr. Lüllman-Rauch: Microscopy (9 pm)

TOTAL EXPECTED EFFORT: 165 pm

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