Ontology highlight
ABSTRACT:
SUBMITTER: Kowalewski B
PROVIDER: S-EPMC3387061 | biostudies-literature | 2012 Jun
REPOSITORIES: biostudies-literature
Proceedings of the National Academy of Sciences of the United States of America 20120611 26
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 arylsulfatase G (ARSG) is the long-sought glucosamine-3-O-sulfatase required to complete the degradation of heparan sulfate. Arsg-deficient mice accumulate heparan sulfate in visceral organs and the cent ...[more]