Volkmar Gieselmann

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Volkmar Gieselmann

PI of Research Project 8:
“Quantitative determination of the effect of transcription factors TFEB and TFE3 on the expression of lysosomal proteins”

 

CONTACT

Prof. Dr. Volkmar Gieselmann

Universität Bonn
Institut für Biochemie und Molekularbiologie
Nussallee 11
D-53115 Bonn
Germany

phone: +49 (0) 228 732411
fax: +49 (0) 228 732416
gieselmann@ibmb.uni-bonn.de
website Gieselmann Lab

 

 

KEY PUBLICATIONS

Comparison of five peptide vectors for improved brain delivery of the lysosomal enzyme arylsulfatase A. Böckenhoff A, Cramer S, Wölte P, Knieling S, Wohlenberg C, Gieselmann V, Galla HJ, Matzner U (2014)  J Neurosci 34:3122-9

Efficacy of enzyme replacement therapy in an aggravated mouse model of metachromatic Leukodystrophy declines with age. Matthes F, Stroobants S, Gerlach D, Wohlenberg C, Wessig C, Fogh J, Gieselmann V, Eckhardt M, D’Hooge R, Matzner U (2012)  Hum Mol Genet 21:2599-609

Absence of 2-hydroxylated sphingolipids is compatible with normal neural development but causes late-onset axon and myelin sheath degeneration. Zöller I, Meixner M, Hartmann D, Büssow H, Meyer R, Gieselmann V, Eckhardt M (2008)  J Neurosci 28:9741-54

Increasing sulfatide synthesis in myelin-forming cells of arylsulfatase A-deficient mice causes demyelination and neurological symptoms reminiscent of human metachromatic leukodystrophy. Ramakrishnan H, Hedayati KK, Lüllmann-Rauch R, Wessig C, Fewou SN, Maier H, Goebel HH, Gieselmann V, Eckhardt M (2007) J Neurosci 27:9482-90

Sulfatide storage in neurons causes hyperexcitability and axonal degeneration in a mouse model of metachromatic leukodystrophy. Eckhardt M, Hedayati KK, Pitsch J, Lüllmann-Rauch R, Beck H, Fewou SN, Gieselmann V (2007)  J Neurosci 27:9009-21

Molecular basis of different forms of metachromatic leukodystrophy. Polten A, Fluharty AL, Fluharty CB, Kappler J, von Figura K, Gieselmann V (1991)  New Engl J Med 324:18-22

Arylsulfatase A pseudodeficiency: Loss of a polyadenylation signal and a N-glycosylation site. Gieselmann V, Kreysing J, Polten A, von Figura K (1989) Proc Natl Acad Sci USA 86:9436-40