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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.

DHTKD1 dimer decarboxylates 2-OA

H+ + Lipo-K110-DLST + 2-OA → CO2 + GlutaryllipoH2-K110-DLST

Reactome: R-HSA-9858321
Compartment: mitochondrial matrix
Rhea: RHEA:69577

Inputs

H+CHEBI:15378, H, mitochondrial matrix
Lipo-K110-DLSTR-HSA-69993, mitochondrial matrix
2-OACHEBI:57499, C6H6O5, mitochondrial matrix

Outputs

CO2CHEBI:16526, CO2, mitochondrial matrix
GlutaryllipoH2-K110-DLSTR-HSA-9858581, mitochondrial matrix

Catalysed by

oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor of OADH complex [mitochondrial matrix]EC 1.2.4

Literature

The mitochondrial 2-oxoadipate and 2-oxoglutarate dehydrogenase complexes share their E2 and E3 components for their function and both generate reactive oxygen species Free Radic Biol Med, 2018
Crystal structure and interaction studies of human DHTKD1 provide insight into a mitochondrial megacomplex in lysine catabolism IUCrJ, 2020
Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex ACS Chem Biol, 2020
A nonsense mutation in DHTKD1 causes Charcot-Marie-Tooth disease type 2 in a large Chinese pedigree Am J Hum Genet, 2012
Genetic basis of alpha-aminoadipic and alpha-ketoadipic aciduria J Inherit Metab Dis, 2015
Heterozygous DHTKD1 Variants in Two European Cohorts of Amyotrophic Lateral Sclerosis Patients Genes (Basel), 2021