Pyruvate dehydrogenase (lipoamide) alpha 2

PDHA2
Identifiers
Aliases	PDHA2, PDHAL, Pyruvate dehydrogenase (lipoamide) alpha 2, pyruvate dehydrogenase alpha 2, pyruvate dehydrogenase E1 alpha 2 subunit
External IDs	HomoloGene: 129539 GeneCards: PDHA2
Gene location (Human) Chr. Chromosome 4 (human) Band 4q22.3 Start 95,840,019 bp End 95,841,474 bp
Gene ontology Molecular function • oxidoreductase activity • pyruvate dehydrogenase (acetyl-transferring) activity • oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor Cellular component • intracellular membrane-bounded organelle • cell nucleus • nucleolus • mitochondrion • mitochondrial matrix Biological process • metabolism • tricarboxylic acid cycle • oxidation-reduction process • acetyl-CoA biosynthetic process from pyruvate • glucose metabolic process • pyruvate metabolic process • regulation of acetyl-CoA biosynthetic process from pyruvate • cellular nitrogen compound metabolic process • carbohydrate metabolic process Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	5161	n/a
Ensembl	ENSG00000163114	n/a
UniProt	P29803	n/a
RefSeq (mRNA)	NM_005390	n/a
RefSeq (protein)	NP_005381	n/a
Location (UCSC)	Chr 4: 95.84 – 95.84 Mb	n/a
PubMed search		n/a

5161

n/a

ENSG00000163114

n/a

P29803

n/a

NM_005390

n/a

NP_005381

n/a

Pyruvate dehydrogenase (lipoamide) alpha 2, also known as pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial or PDHE1-A type II, is an enzyme that in humans is encoded by the PDHA2 gene.

Two mature PDHA proteins come together with two PDHB proteins to form a heterotetrameric E1 subunit. Crystal Structures allowed for a model in which the enzyme undergoes a 2-A shuttle-like motion of its heterodimers to perform the catalysis. The protein encoded by the human PDHA2 gene is part of the pyruvate dehydrogenase multienzyme complex. The entire human complex is 9.5 MDa in size, and has been described as 60-meric, meaning there are over 60 components that are assembled to make the entire complex. These subunits are conserved across many species, as the function of this complex is essential for the generation of ATP for all eukaryotes. Each component is responsible for the catalysis of one step in this pathway; this complex exists for the purpose of channeling the intermediates of each reaction to the next enzyme, thus greatly increasing the rate of reaction.

The pyruvate dehydrogenase complex is responsible for the oxidative decarboxylation of pyruvate, with the final product being Acetyl CoA. Overall the complex catalyzes five reactions, with the overall reaction being:

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