Pyruvate dehydrogenase (lipoamide) alpha 1

PDHA1
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1NI4, 2OZL, 3EXE, 3EXF, 3EXG, 3EXH, 3EXI
Identifiers
Aliases	PDHA1, PDHA, PDHCE1A, PHE1A, PDHAD, Pyruvate dehydrogenase (lipoamide) alpha 1, pyruvate dehydrogenase alpha 1, pyruvate dehydrogenase E1 alpha 1 subunit, pyruvate dehydrogenase E1 subunit alpha 1
External IDs	OMIM: 300502; MGI: 97532; HomoloGene: 121491; GeneCards: PDHA1; OMA:PDHA1 - orthologs
Gene location (Human)
Chr.	X chromosome (human)
End	19,361,718 bp
Gene location (Mouse)
Chr.	X chromosome (mouse)
End	158,921,409 bp
RNA expression pattern
	Top expressed in
	apex of heart; ; left ventricle; ; right auricle of heart; ; gastrocnemius muscle; ; muscle of thigh; ; mucosa of transverse colon; ; rectum; ; body of stomach; ; body of pancreas; ; right ventricle;
	Top expressed in
	right ventricle; ; digastric muscle; ; sternocleidomastoid muscle; ; temporal muscle; ; extraocular muscle; ; myocardium of ventricle; ; triceps brachii muscle; ; brown adipose tissue; ; soleus muscle; ; interventricular septum;
	More reference expression data
	; ;
	More reference expression data
Gene ontology
Molecular function	pyruvate dehydrogenase activity; oxidoreductase activity; pyruvate dehydrogenase (acetyl-transferring) activity; oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor; pyruvate dehydrogenase (NAD+) activity; protein binding;
Cellular component	intracellular membrane-bounded organelle; myelin sheath; pyruvate dehydrogenase complex; mitochondrial matrix; nucleus; nucleolus; mitochondrion;
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; acetyl-CoA biosynthetic process from pyruvate; cellular nitrogen compound metabolic process; carbohydrate metabolic process;
	Sources:Amigo / QuickGO
Orthologs
	5160
	18597
	ENSG00000131828
	ENSMUSG00000031299
	P08559; Q5JPT9
	P35486
	NM_000284; NM_001173454; NM_001173455; NM_001173456
	NM_008810
	NP_000275; NP_001166925; NP_001166926; NP_001166927
	NP_032836
	Wikidata
View/Edit Human	View/Edit Mouse

Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial is an enzyme that in humans is encoded by the PDHA1 gene.The pyruvate dehydrogenase complex is a nuclear-encoded mitochondrial matrix multienzyme complex that provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. The PDH complex is composed of multiple copies of 3 enzymes: E1 (PDHA1); dihydrolipoyl transacetylase (DLAT) (E2; EC 2.3.1.12); and dihydrolipoyl dehydrogenase (DLD) (E3; EC 1.8.1.4). The E1 enzyme is a heterotetramer of 2 alpha and 2 beta subunits. The E1-alpha subunit contains the E1 active site and plays a key role in the function of the PDH complex.