Institute of Biochemistry and Molecular Biology
University of Bonn
D- 53115 Bonn
Phone +49 (0) 228 73-2411
Fax: +49 (0) 228 73-2416
Curriculum vitae Professor Dr. Volkmar Gieselmann
The group is interested in lysosomal shingolipid storage diseases which mainly affect myelin. Lipid storage is caused by deficiencies of lysosomal enzymes which are involved in the degradation pathways of these sphingolipids. Disease in the focus of the group are metachromatic leukodystrophy and Krabbe disease. We try to understand how storage of lipids affects function of the myelin producing oligodendrocytes and Schwann cells. The second major interest in the development of therapies for these devastating and so far incurable diseases.
The group runs a mass spectrometry facility and offers a broad range of mass spectrometry based techniques in particular for protein analysis and proteomics.
5 most important publications
1. Ramakrishnan H, Hedayati KK, Lüllmann-Rauch R, Wessig C, Fewou SN, Maier H, Goebel HH, Gieselmann V, Eckhardt M. (2007) Increasing sulfatide synthesis in myelin-forming cells of arylsulfatase A deficient mice causes demyelination and neurological symptoms reminiscent of human metachromatic leukodystrophy. J Neurosci, 27: 9482-9490.
2. Matzner U, Herbst E, Hedayati KK, Lüllmann-Rauch RR, Wessig C, Schröder S, Eistrup C, Möller C, Fogh J, Gieselmann V. (2005) Enzyme replacement improves nervous system pathology and function in a mouse model for metachromatic leukodystrophy. Hum Mol Genet, 14: 1139-1152.
3. Hess, B, Saftig P, Hartmann D, Coenen R, Lüllmann Rauch R, Goebel HH, Evers M, von Figura K, D’Hogge R, Nagels G, DeDeyn P, Peters C, ieselmann V. (1996). Phenotype of arylsulfatase A deficient mice: relationship to human metachromatic leukodystrophy. PNAS USA, 93: 14821-14826.
4. Polten A, Fluharty AL, Fluharty CB, Kappler, J, von Figura K, Gieselmann V. (1991) The molecular basis of different forms of Metachromatic Leukodystrophy. New Engl J Med, 324: 18-22.
5. Gieselmann V, Polten A, Kreysing J, von Figura K. (1989), Arylsulfatase A pseudodeficiency: loss of a polyadenylation signal and a N-glycosylation site. PNAS USA, 86: 9436-9440.