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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.

4,4-dimethylcholesta-8(9),14,24-trien-3beta-ol is reduced to 4,4-dimethylcholesta-8(9),24-dien-3beta-ol [LBR]

4,4DMCHtOL + NADPH + H+ → 14DMLANOL + NADP+

Reactome: R-HSA-194674
Compartment: nuclear envelope
Rhea: RHEA:18563

Inputs

4,4DMCHtOLCHEBI:17813, C29H46O, nuclear envelope
NADPHCHEBI:16474, C21H30N7O17P3, nuclear envelope
H+CHEBI:15378, H, nuclear envelope

Outputs

14DMLANOLCHEBI:18364, C29H48O, nuclear envelope
NADP+CHEBI:18009, C21H29N7O17P3, nuclear envelope

Catalysed by

Delta14-sterol reductase activity of LBR [nuclear envelope]EC 1.3.1.70; UniProt Q14739

Literature

Autosomal recessive HEM/Greenberg skeletal dysplasia is caused by 3 beta-hydroxysterol delta 14-reductase deficiency due to mutations in the lamin B receptor gene Am J Hum Genet, 2003