[HTML][HTML] Saposin B-dependent reconstitution of arylsulfatase A activity in vitro and in cell culture models of metachromatic leukodystrophy
U Matzner, B Breiden, G Schwarzmann… - Journal of biological …, 2009 - ASBMB
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 results in a block of sulfatide degradation,
progressive intralysosomal accumulation of sulfatide, and the fatal lysosomal storage
disease metachromatic leukodystrophy. We studied the coupled Sap B-ASA reaction in vitro …
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 results in a block of sulfatide degradation,
progressive intralysosomal accumulation of sulfatide, and the fatal lysosomal storage
disease metachromatic leukodystrophy. We studied the coupled Sap B-ASA reaction in vitro …