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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

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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. .

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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.

ASAH1 hydrolyzes ceramide

CERA + H2O → SPG + RCOO-

Reactome: R-HSA-1606602
Compartment: lysosomal lumen
Rhea: RHEA:78456

Inputs

CERACHEBI:83273, C4H7NO3R2, lysosomal lumen
H2OCHEBI:15377, H2O, lysosomal lumen

Outputs

SPGCHEBI:84410, C3H9NO2R, lysosomal lumen
RCOO-CHEBI:28868, CO2R, lysosomal lumen

Catalysed by

N-acylsphingosine amidohydrolase activity of ASAH1 [lysosomal lumen]EC 3.5.1.23

Literature

Purification, characterization, and biosynthesis of human acid ceramidase J Biol Chem, 1995
Human acid ceramidase gene: novel mutations in Farber disease Mol Genet Metab, 2000
Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification Of the first molecular lesion causing Farber disease J Biol Chem, 1996
Structural and membrane-binding properties of saposin D Eur J Biochem, 1999
Structural basis for the activation of acid ceramidase Nat Commun, 2018