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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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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.

phytanoyl-CoA + 2-oxoglutarate + O2 => 2-hydroxyphytanoyl-CoA + succinate + CO2

Phytanoyl-CoA + 2OG + O2 → 3S2HPhy-CoA + SUCCA + CO2

Reactome: R-HSA-389639
Compartment: peroxisomal matrix
Rhea: RHEA:16066

Inputs

Phytanoyl-CoACHEBI:57391, C41H70N7O17P3S, peroxisomal matrix
2OGCHEBI:16810, C5H4O5, peroxisomal matrix
O2CHEBI:15379, O2, peroxisomal matrix

Outputs

3S2HPhy-CoACHEBI:57334, C41H70N7O18P3S, peroxisomal matrix
SUCCACHEBI:30031, C4H4O4, peroxisomal matrix
CO2CHEBI:16526, CO2, peroxisomal matrix

Catalysed by

phytanoyl-CoA dioxygenase activity of PHYH:Fe++ [peroxisomal matrix]EC 1.14.11.18

Literature

Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease J Biol Chem, 2005
Structure-function analysis of phytanoyl-CoA 2-hydroxylase mutations causing Refsum's disease Hum Mol Genet, 2001