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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.

SMPD1 hydrolyzes SPHM

SPHM d18:1 + H2O → CERA + ChoP + H+

Reactome: R-HSA-1605797
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

SPHM d18:1CHEBI:17636, C24H48N2O6PR, lysosomal lumen
H2OCHEBI:15377, H2O, lysosomal lumen

Outputs

CERACHEBI:83273, C4H7NO3R2, lysosomal lumen
ChoPCHEBI:295975, C5H13NO4P, lysosomal lumen
H+CHEBI:15378, H, lysosomal lumen

Catalysed by

sphingomyelin phosphodiesterase activity of SMPD1 [lysosomal lumen]EC 3.1.4.12; UniProt P17405

Literature

Human acid sphingomyelinase. Isolation, nucleotide sequence and expression of the full-length and alternatively spliced cDNAs J Biol Chem, 1991
Structural organization and complete nucleotide sequence of the gene encoding human acid sphingomyelinase (SMPD1) Genomics, 1992
Molecular basis of acid sphingomyelinase deficiency in a patient with Niemann-Pick disease type A Biochem Biophys Res Commun, 1991
Identification and characterization of SMPD1 mutations causing Niemann-Pick types A and B in Spanish patients Hum Mutat, 2009