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Volkmar Gieselmann

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Articles 119
Citations 2301
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Recent Articles
1.
Kaade E, Mausbach S, Erps N, Sylvester M, Shakeri F, Jachimowicz R, et al.
Sci Rep . 2025 Jan; 15(1):3638. PMID: 39880911
No abstract available.
2.
Edgar K, Kaade E, Simone M, Mausbach S, Nina E, Erps N, et al.
Sci Rep . 2024 Nov; 14(1):28296. PMID: 39550382
Lysosomes play a crucial role in metabolic adaptation to starvation, but detailed in vivo studies are scarce. Therefore, we investigated the changes of the proteome of liver lysosomes in mice...
3.
Yaghootfam C, Sylvester M, Turk B, Gieselmann V, Matzner U
Mol Ther . 2023 Aug; 31(10):2962-2974. PMID: 37644722
A deficiency of human arylsulfatase A (hASA) causes metachromatic leukodystrophy (MLD), a lysosomal storage disease characterized by sulfatide accumulation and central nervous system (CNS) demyelination. Efficacy of enzyme replacement therapy...
4.
Hardt R, Dehghani A, Schoor C, Godderz M, Cengiz Winter N, Ahmadi S, et al.
Cell Mol Life Sci . 2023 Aug; 80(9):260. PMID: 37594553
Oligodendrocytes are generated via a two-step mechanism from pluripotent neural stem cells (NSCs): after differentiation of NSCs to oligodendrocyte precursor/NG2 cells (OPCs), they further develop into mature oligodendrocytes. The first...
5.
Singh J, Elhabashy H, Muthukottiappan P, Stepath M, Eisenacher M, Kohlbacher O, et al.
Nat Commun . 2022 Oct; 13(1):6212. PMID: 36266287
Lysosomes are well-established as the main cellular organelles for the degradation of macromolecules and emerging as regulatory centers of metabolism. They are of crucial importance for cellular homeostasis, which is...
6.
Laugwitz L, Santhanakumaran V, Spieker M, Boehringer J, Bender B, Gieselmann V, et al.
JIMD Rep . 2022 Jul; 63(4):292-302. PMID: 35822086
Metachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal storage disease caused by deficiency of arylsulfatase A (ARSA). Heterozygous carriers of disease-causing variants and individuals harbouring pseudodeficiency alleles in the gene...
7.
Tuermer A, Mausbach S, Kaade E, Damme M, Sylvester M, Gieselmann V, et al.
Proteomics . 2021 Aug; 21(19):e2100043. PMID: 34432360
Neuronal ceroid lipofuscinoses (NCLs) collectively account for the highest prevalence of inherited neurodegenerative diseases in childhood. This disease group is classified by the deposition of similar autofluorescence storage material in...
8.
Yaghootfam C, Gehrig B, Sylvester M, Gieselmann V, Matzner U
J Biol Chem . 2021 Aug; 297(3):101064. PMID: 34375644
An inherited deficiency of arylsulfatase A (ASA) causes the lysosomal storage disease metachromatic leukodystrophy (MLD) characterized by massive intralysosomal storage of the acidic glycosphingolipid sulfatide and progressive demyelination. Lyso-sulfatide, which...
9.
Singh J, Ponnaiyan S, Gieselmann V, Winter D
Anal Chem . 2021 Feb; 93(8):3762-3769. PMID: 33591729
Chemical cross-linking has become a powerful tool for the analysis of protein structures and interactions by mass spectrometry. A particular strength of this approach is the ability to investigate native...
10.
Kaminski D, Yaghootfam C, Matthes F, Ressing A, Gieselmann V, Matzner U
Hum Mol Genet . 2020 Dec; 29(23):3807-3817. PMID: 33367737
Enzyme replacement therapies, allogeneic bone marrow transplantation and gene therapies are treatment options for lysosomal storage diseases caused by inherited deficiencies of soluble lysosomal enzymes. Independent from the approach, the...