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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

Data

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Human metabolism

Reactome's human metabolism drawn as one network. Each molecule has one home node; widely shared molecules also appear as dashed copies in other territories, linked from their details. .

Territories

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Metabolite; its structure appears as you zoom in
Reaction, arrow toward the product
Cofactors and co-substrates are written beside the reaction when you zoom in
Dashed ring: a copy of a widely shared molecule or cofactor in another territory or pathway
Compartment transfer of the molecule it is linked to
Binding, release or complex event; its proteins are named in its details
Outline: reactions mostly in the mitochondrion

Arrows follow Reactome's curated reaction direction.

GALC hydrolyzes GalCer

H2O + GalCer → CERA + Glc

Reactome: R-HSA-1606564
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

H2OCHEBI:15377, H2O, lysosomal lumen
GalCerCHEBI:36498, C10H17NO8R2, lysosomal lumen

Outputs

CERACHEBI:83273, C4H7NO3R2, lysosomal lumen
GlcCHEBI:4167, C6H12O6, lysosomal lumen

Catalysed by

galactosylceramidase activity of GALC [lysosomal lumen]EC 3.2.1.46; UniProt P54803

Literature

Purification and characterization of galactocerebrosidase from human lymphocytes J Biochem, 1994
Molecular genetics of Krabbe disease (globoid cell leukodystrophy): diagnostic and clinical implications Hum Mutat, 1997
Cloning and expression of cDNA encoding human galactocerebrosidase, the enzyme deficient in globoid cell leukodystrophy Hum Mol Genet, 1993
Galactocerebrosidase from human urine: purification and partial characterization Biochim Biophys Acta, 1993
Saposin A: second cerebrosidase activator protein Proc Natl Acad Sci U S A, 1989
A mutation in the saposin A coding region of the prosaposin gene in an infant presenting as Krabbe disease: first report of saposin A deficiency in humans Mol Genet Metab, 2005