GLA hydrolyzes PSAP(195-273):Gal2Cer:PE
H2O + PSAP(195-273):Gal2Cer:PE → Gal + GalCer + PSAP(195-273) dimer + PE 30,32,34
- Reactome
- R-HSA-9841189
- Compartment
- lysosomal lumen
- Rhea
- None recorded
Inputs
- H2OCHEBI:15377, H2O, lysosomal lumen
- PSAP(195-273):Gal2Cer:PER-HSA-9841186, lysosomal lumen
Outputs
- GalCHEBI:28061, C6H12O6, lysosomal lumen
- GalCerCHEBI:36498, C10H17NO8R2, lysosomal lumen
- PSAP(195-273) dimerR-HSA-9840482, lysosomal lumen
- PE 30,32,34R-ALL-9840939, lysosomal lumen
Catalysed by
- alpha-galactosidase activity of GLA dimer [lysosomal lumen]EC 3.2.1.22
Literature
- Human alpha-galactosidase A: nucleotide sequence of a cDNA clone encoding the mature enzyme Proc Natl Acad Sci U S A, 1986
- The molecular defect leading to Fabry disease: structure of human alpha-galactosidase J Mol Biol, 2004
- Nature and frequency of mutations in the alpha-galactosidase A gene that cause Fabry disease Am J Hum Genet, 1993
- Fabry disease: identification of 50 novel alpha-galactosidase A mutations causing the classic phenotype and three-dimensional structural analysis of 29 missense mutations Hum Genomics, 2006
- Prosaposin deficiency: further characterization of the sphingolipid activator protein-deficient sibs. Multiple glycolipid elevations (including lactosylceramidosis), partial enzyme deficiencies and ultrastructure of the skin in this generalized sphingolipid storage disease Hum Genet, 1993
- Studies on human liver alpha-galactosidases. I. Purification of alpha-galactosidase A and its enzymatic properties with glycolipid and oligosaccharide substrates J Biol Chem, 1979