Pyruvate dehydrogenase (lipoamide) beta

PDHB
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1NI4, 2OZL, 3EXE, 3EXF, 3EXG, 3EXH, 3EXI
Identifiers
Aliases	PDHB, PDHBD, PDHE1-B, PHE1B, Pyruvate dehydrogenase (lipoamide) beta, PDHE1B, pyruvate dehydrogenase E1 beta subunit, pyruvate dehydrogenase E1 subunit beta
External IDs	OMIM: 179060; MGI: 1915513; HomoloGene: 712; GeneCards: PDHB; OMA:PDHB - orthologs
Gene location (Human)
Chr.	Chromosome 3 (human)
End	58,433,857 bp
Gene location (Mouse)
Chr.	Chromosome 14 (mouse)
End	14,303,777 bp
RNA expression pattern
	Top expressed in
	endothelial cell; ; right ventricle; ; biceps brachii; ; Skeletal muscle tissue of rectus abdominis; ; Skeletal muscle tissue of biceps brachii; ; myocardium of left ventricle; ; body of tongue; ; deltoid muscle; ; Epithelium of choroid plexus; ; vastus lateralis muscle;
	Top expressed in
	spermatocyte; ; muscle of thigh; ; neural layer of retina; ; yolk sac; ; spermatid; ; right kidney; ; morula; ; dentate gyrus of hippocampal formation granule cell; ; superior frontal gyrus; ; lip;
	More reference expression data
	n/a
Gene ontology
Molecular function	pyruvate dehydrogenase activity; protein binding; catalytic activity; oxidoreductase activity; pyruvate dehydrogenase (acetyl-transferring) activity; pyruvate dehydrogenase (NAD+) activity;
Cellular component	pyruvate dehydrogenase complex; nucleoplasm; mitochondrial matrix; mitochondrion; extracellular exosome; nucleus;
Biological process	mitochondrial acetyl-CoA biosynthetic process from pyruvate; tricarboxylic acid cycle; regulation of acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process; glucose metabolic process; metabolism; cellular nitrogen compound metabolic process; carbohydrate metabolic process; acetyl-CoA biosynthetic process from pyruvate;
	Sources:Amigo / QuickGO
Orthologs
	5162
	68263
	ENSG00000168291
	ENSMUSG00000021748
	P11177
	Q9D051
	NM_000925; NM_001173468; NM_001315536
	NM_024221
	NP_000916; NP_001166939; NP_001302465
	NP_077183
	Wikidata
View/Edit Human	View/Edit Mouse

Pyruvate dehydrogenase (lipoamide) beta, also known as pyruvate dehydrogenase E1 component subunit beta, mitochondrial or PDHE1-B is an enzyme that in humans is encoded by the PDHB gene. The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO₂, and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 beta subunit. Mutations in this gene are associated with pyruvate dehydrogenase E1-beta deficiency.