Saposin B-dependent Reconstitution of Arylsulfatase A Activity in Vitro and in Cell Culture Models of Metachromatic Leukodystrophy

Arylsulfatase A (ASA) catalyzes the intralysosomal desulfation of 3-O-sulfogalactosylceramide (sulfatide) to galactosylceramide. The reaction requires saposin B (Sap B), a non-enzymatic proteinaceous cofactor which presents sulfatide to the catalytic site of ASA. The lack of either ASA or Sap B resu...

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Hlavní autoři: Matzner, Ulrich, Breiden, Bernadette, Schwarzmann, Günter, Yaghootfam, Afshin, Fluharty, Arvan L., Hasilik, Andrej, Sandhoff, Konrad, Gieselmann, Volkmar
Médium: Artigo
Jazyk:Inglês
Vydáno: American Society for Biochemistry and Molecular Biology 2009
Témata:
Lipids and Lipoproteins: Metabolism, Regulation, and Signaling
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC2666589/
https://ncbi.nlm.nih.gov/pubmed/19224915
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M809457200
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