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Identification
HMDB Protein ID HMDBP00050
Secondary Accession Numbers
  • 5279
Name Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
Synonyms
  1. PDHE1-A type II
Gene Name PDHA2
Protein Type Enzyme
Biological Properties
General Function Involved in oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
Specific Function The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.
Pathways
  • Butanoate metabolism
  • Citrate cycle (TCA cycle)
  • Glycolysis / Gluconeogenesis
  • HIF-1 signaling pathway
  • Pyruvate metabolism
Reactions
Pyruvic acid + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine → [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO(2) details
Pyruvic acid + Thiamine pyrophosphate → 2-(a-Hydroxyethyl)thiamine diphosphate + Carbon dioxide details
Pyruvic acid + Enzyme N6-(lipoyl)lysine → [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + Carbon dioxide details
2-(a-Hydroxyethyl)thiamine diphosphate + Enzyme N6-(lipoyl)lysine → [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + Thiamine pyrophosphate details
GO Classification
Biological Process
glycolysis
pyruvate metabolic process
Cellular Component
mitochondrial matrix
Component
organelle
membrane-bounded organelle
intracellular membrane-bounded organelle
Function
catalytic activity
oxidoreductase activity
pyruvate dehydrogenase activity
pyruvate dehydrogenase (acetyl-transferring) activity
oxidoreductase activity, acting on the aldehyde or oxo group of donors
oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
Molecular Function
pyruvate dehydrogenase (acetyl-transferring) activity
Process
metabolic process
small molecule metabolic process
alcohol metabolic process
monosaccharide metabolic process
hexose metabolic process
glucose metabolic process
oxidation reduction
glucose catabolic process
glycolysis
Cellular Location
  1. Mitochondrion matrix
Gene Properties
Chromosome Location 4
Locus 4q22-q23
SNPs PDHA2
Gene Sequence
>1167 bp
ATGCTGGCCGCCTTCATCTCCCGCGTGTTGAGGCGAGTTGCCCAGAAATCAGCTCGCAGA
GTGCTGGTGGCATCCCGTAACTCCTCAAATGACGCTACATTTGAAATTAAGAAATGTGAT
CTTTATCTGTTGGAAGAGGGTCCCCCTGTCACTACAGTGCTCACTAGGGCGGAGGGGCTT
AAATACTACAGGATGATGCTGACTGTTCGCCGCATGGAATTGAAGGCAGATCAGCTGTAC
AAACAGAAATTCATTCGCGGTTTCTGTCACCTGTGCGATGGTCAGGAAGCTTGTTGCGTG
GGCCTTGAGGCCGGCATAAACCCCTCGGATCACGTCATTACATCCTATAGGGCTCATGGT
GTGTGCTATACTCGGGGACTTTCTGTCCGATCCATTCTCGCAGAGCTGACGGGAAGAAGA
GGAGGTTGTGCTAAAGGAAAAGGAGGATCGATGCATATGTATACCAAGAACTTCTATGGG
GGCAATGGCATCGTCGGTGCACAGGGCCCCCTGGGCGCTGGCATTGCTCTGGCCTGTAAA
TATAAAGGAAACGATGAGATCTGTTTGACTTTATATGGGGATGGCGCTGCGAATCAGGGG
CAGATAGCCGAAGCTTTCAATATGGCAGCTTTATGGAAATTACCTTGTGTTTTCATCTGT
GAGAATAACCTATATGGAATGGGAACATCTACTGAGAGAGCAGCAGCCAGCCCTGATTAC
TACAAGAGGGGCAATTTTATCCCTGGGCTAAAGGTCGATGGAATGGATGTTCTGTGTGTT
CGTGAGGCAACAAAATTTGCAGCTAACTACTGTAGATCTGGAAAGGGGCCCATACTGATG
GAGCTGCAAACCTACCGTTATCATGGACACAGTATGAGTGATCCTGGAGTCAGTTATCGT
ACACGAGAAGAAATTCAGGAAGTAAGAAGTAAGAGGGATCCTATAATAATTCTCCAAGAT
AGAATGGTAAACAGCAAGCTCGCCACTGTGGAAGAATTAAAGGAAATTGGGGCTGAGGTG
AGGAAAGAAATTGATGATGCTGCCCAGTTTGCTACCACTGATCCTGAGCCACATTTGGAA
GAATTAGGCCATCACATCTACAGCAGTGATTCATCTTTTGAAGTTCGTGGTGCAAATCCA
TGGATCAAGTTTAAGTCCGTCAGTTAA
Protein Properties
Number of Residues 388
Molecular Weight 42932.855
Theoretical pI 8.46
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
MLAAFISRVLRRVAQKSARRVLVASRNSSNDATFEIKKCDLYLLEEGPPVTTVLTRAEGL
KYYRMMLTVRRMELKADQLYKQKFIRGFCHLCDGQEACCVGLEAGINPSDHVITSYRAHG
VCYTRGLSVRSILAELTGRRGGCAKGKGGSMHMYTKNFYGGNGIVGAQGPLGAGIALACK
YKGNDEICLTLYGDGAANQGQIAEAFNMAALWKLPCVFICENNLYGMGTSTERAAASPDY
YKRGNFIPGLKVDGMDVLCVREATKFAANYCRSGKGPILMELQTYRYHGHSMSDPGVSYR
TREEIQEVRSKRDPIIILQDRMVNSKLATVEELKEIGAEVRKEIDDAAQFATTDPEPHLE
ELGHHIYSSDSSFEVRGANPWIKFKSVS
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID P29803
UniProtKB/Swiss-Prot Entry Name ODPAT_HUMAN
PDB IDs Not Available
GenBank Gene ID AK313872
GeneCard ID PDHA2
GenAtlas ID PDHA2
HGNC ID HGNC:8807
References
General References
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