Reaction: bHEXA,bHEXS hydrolyze GM2A:SM2
- in pathway: Glycosphingolipid catabolism
The beta-hexosaminidase isoforms A (bHEXA) and S (bHEXS) contain an alpha subunit (HEXA) that cleaves negatively charged substrates. Each of bHEXA and bHEXS hydrolyze the charged sulfated glycosphingolipid SM2 to form SM3. Under lysosomal conditions the reaction takes place only in the presence of both BMP (lysobisphosphatidate) and GM2AP (Hepbildikler et al., 2002).
Reaction - small molecule participants:
SM3 [lysosomal lumen]
GalNAc [lysosomal lumen]
H2O [lysosomal lumen]
Reactome.org reaction link: R-HSA-9840884
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Reaction input - small molecules:
water
Reaction output - small molecules:
beta-D-3-sulfogalactosyl-(1->4)-beta-D-glucosyl-(1<->1')-ceramide(1-)
N-acetyl-D-galactosamine
Reactome.org link: R-HSA-9840884