Arylsulfatase G inactivation causes loss of heparan sulfate 3-O-sulfatase activity and mucopolysaccharidosis in mice

Deficiency of glycosaminoglycan (GAG) degradation causes a subclass of lysosomal storage disorders called mucopolysaccharidoses (MPSs), many of which present with severe neuropathology. Critical steps in the degradation of the GAG heparan sulfate remain enigmatic. Here we show that the lysosomal ary...

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Detalhes bibliográficos
Main Authors: Kowalewski, Björn, Lamanna, William C., Lawrence, Roger, Damme, Markus, Stroobants, Stijn, Padva, Michael, Kalus, Ina, Frese, Marc-André, Lübke, Torben, Lüllmann-Rauch, Renate, D’Hooge, Rudi, Esko, Jeffrey D., Dierks, Thomas
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2012
Assuntos:
Biological Sciences
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3387061/
https://ncbi.nlm.nih.gov/pubmed/22689975
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1202071109
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