Loading the map…

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

Select a territory to bring it forward.

Reading the map

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.

ELOVL1,4 elongate TCS-CoA and Mal-CoA to 3OHC-CoA

TCS-CoA + Mal-CoA + H+ → 3OHC-CoA + CoA-SH + CO2

Reactome: R-HSA-548830
Compartment: cytosol · endoplasmic reticulum membrane
Rhea: RHEA:36516

Inputs

TCS-CoACHEBI:65052, C45H78N7O17P3S, cytosol
Mal-CoACHEBI:57384, C24H33N7O19P3S, cytosol
H+CHEBI:15378, H, cytosol

Outputs

3OHC-CoACHEBI:73980, C47H80N7O18P3S, cytosol
CoA-SHCHEBI:57287, C21H32N7O16P3S, cytosol
CO2CHEBI:16526, CO2, cytosol

Catalysed by

fatty acid elongase activity of ELOVL1,4 [endoplasmic reticulum membrane]EC 2.3.1.199

Literature

Role of Stargardt-3 macular dystrophy protein (ELOVL4) in the biosynthesis of very long chain fatty acids Proc Natl Acad Sci U S A, 2008
Dominant negative mechanism underlies autosomal dominant Stargardt-like macular dystrophy linked to mutations in ELOVL4 J Biol Chem, 2005