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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.

HPDL dioxygenates HPPA

HPP + O2 → 4-HMA + CO2

Reactome: R-HSA-9856718
Compartment: mitochondrial matrix
Rhea: RHEA:21377

Inputs

HPPCHEBI:36242, C9H7O4, mitochondrial matrix
O2CHEBI:15379, O2, mitochondrial matrix

Outputs

4-HMACHEBI:17210, C8H7O4, mitochondrial matrix
CO2CHEBI:16526, CO2, mitochondrial matrix

Catalysed by

4-hydroxymandelate synthase activity of HPDL:Fe2+ [mitochondrial matrix]EC 1.13.11.46

Literature

Biosynthesis, Deficiency, and Supplementation of Coenzyme Q Antioxidants (Basel), 2023
The polar oxy-metabolome reveals the 4-hydroxymandelate CoQ10 synthesis pathway Nature, 2021
Bi-allelic HPDL Variants Cause a Neurodegenerative Disease Ranging from Neonatal Encephalopathy to Adolescent-Onset Spastic Paraplegia Am J Hum Genet, 2020