Analysis of the Biogenesis of Heparan Sulfate Acetyl-CoA:α-Glucosaminide N-Acetyltransferase Provides Insights into the Mechanism Underlying Its Complete Deficiency in Mucopolysaccharidosis IIIC

Heparan sulfate acetyl-CoA:α-glucosaminide N-acetyltransferase (HGSNAT) catalyzes the transmembrane acetylation of heparan sulfate in lysosomes required for its further catabolism. Inherited deficiency of HGSNAT in humans results in lysosomal storage of heparan sulfate and causes the severe neurodeg...

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Hlavní autoři: Durand, Stéphanie, Feldhammer, Matthew, Bonneil, Éric, Thibault, Pierre, Pshezhetsky, Alexey V.
Médium: Artigo
Jazyk:Inglês
Vydáno: American Society for Biochemistry and Molecular Biology 2010
Témata:
Enzymology
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC2951197/
https://ncbi.nlm.nih.gov/pubmed/20650889
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M110.141150
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