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MS
unreviewed

alpha-Galactosidase A (GLA)

1 reactions · 1 pathways

Clinical / pharmacological. Fabry disease (X-linked)

What it does, reaction by reaction

1 reactions

Sphingolipid Degradation (Lysosomal) Lipid Metabolism · Lysosome

step 3 Irreversible/directional

Globotriaosylceramide (Gb3) -> lactosylceramide + galactose

Converts Globotriaosylceramide into Lactosylceramide Galactose

Notes

X-linked deficiency causes Fabry disease (angiokeratomas, neuropathic pain, renal failure).

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.

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