Reaction: ARSA removes sulfate from SM3
- in pathway: Glycosphingolipid catabolism
Sulfated sphingoglycolipid GM3 (SM3, sulfated LacCer, dihexosyl sulfatide) is desulfated by arylsulfatase A (ARSA) to lactosylceramide (LacCer). In metachromatic leukodystrophy (MLD, MIM:250100), SM3 and other sulfatides accumulate, usually because of ARSA deficiency (Philppart et al., 1971; Whitfield et al., 2001).
Reaction - small molecule participants:
LacCer [lysosomal lumen]
SO4(2-) [lysosomal lumen]
H2O [lysosomal lumen]
SM3 [lysosomal lumen]
Reactome.org reaction link: R-HSA-9840949
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Reaction input - small molecules:
water
beta-D-3-sulfogalactosyl-(1->4)-beta-D-glucosyl-(1<->1')-ceramide(1-)
Reaction output - small molecules:
beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-N-acylsphingoid
sulfate
Reactome.org link: R-HSA-9840949