unreviewed
beta-Hexosaminidase A (HEXA) + GM2 activator
1 reactions · 1 pathways
Clinical / pharmacological. Tay-Sachs disease
What it does, reaction by reaction
1 reactions
Sphingolipid Degradation (Lysosomal) Lipid Metabolism · Lysosome
step 2
Irreversible/directional
GM2 ganglioside -> GM3 ganglioside + N-acetylgalactosamine
Converts GM2 ganglioside into GM3 ganglioside N-acetylgalactosamine
› Notes
HEXA deficiency causes Tay-Sachs disease (cherry-red macula, no hepatosplenomegaly); HEXA+HEXB deficiency causes Sandhoff disease.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
0 entries
No regulation recorded for this enzyme in the source documents.
Recent literature
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