Tmic
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NamePyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
Synonyms
  • 1.2.4.1
  • PDHE1-A type I
  • PHE1A
Gene NamePDHA1
OrganismHuman
Amino acid sequence
>lcl|BSEQ0001140|Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
MRKMLAAVSRVLSGASQKPASRVLVASRNFANDATFEIKKCDLHRLEEGPPVTTVLTRED
GLKYYRMMQTVRRMELKADQLYKQKIIRGFCHLCDGQEACCVGLEAGINPTDHLITAYRA
HGFTFTRGLSVREILAELTGRKGGCAKGKGGSMHMYAKNFYGGNGIVGAQVPLGAGIALA
CKYNGKDEVCLTLYGDGAANQGQIFEAYNMAALWKLPCIFICENNRYGMGTSVERAAAST
DYYKRGDFIPGLRVDGMDILCVREATRFAAAYCRSGKGPILMELQTYRYHGHSMSDPGVS
YRTREEIQEVRSKSDPIMLLKDRMVNSNLASVEELKEIDVEVRKEIEDAAQFATADPEPP
LEELGYHIYSSDPPFEVRGANQWIKFKSVS
Number of residues390
Molecular Weight43295.255
Theoretical pI8.14
GO Classification
Functions
  • pyruvate dehydrogenase (acetyl-transferring) activity
  • pyruvate dehydrogenase activity
Processes
  • small molecule metabolic process
  • glucose metabolic process
  • cellular metabolic process
  • acetyl-CoA biosynthetic process from pyruvate
  • pyruvate metabolic process
  • regulation of acetyl-CoA biosynthetic process from pyruvate
  • tricarboxylic acid cycle
Components
  • mitochondrion
  • pyruvate dehydrogenase complex
  • nucleus
  • mitochondrial matrix
  • myelin sheath
General FunctionPyruvate dehydrogenase activity
Specific FunctionThe 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.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID387009
UniProtKB IDP08559
UniProtKB Entry NameODPA_HUMAN
Cellular LocationMitochondrion matrix
Gene sequence
>lcl|BSEQ0018980|Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1)
ATGAGGAAGATGCTCGCCGCCGTCTCCCGCGTGCTGTCTGGCGCTTCTCAGAAGCCGGCA
AGCAGAGTGCTGGTAGCATCCCGTAATTTTGCAAATGATGCTACATTTGAAATTAAGAAA
TGTGACCTTCACCGGCTGGAAGAAGGCCCTCCTGTCACAACAGTGCTCACCAGGGAGGAT
GGGCTCAAATACTACAGGATGATGCAGACTGTACGCCGAATGGAGTTGAAAGCAGATCAG
CTGTATAAACAGAAAATTATTCGTGGTTTCTGTCACTTGTGTGATGGTCAGGAAGCTTGC
TGTGTGGGCCTGGAGGCCGGCATCAACCCCACAGACCATCTCATCACAGCCTACCGGGCT
CACGGCTTTACTTTCACCCGGGGCCTTTCCGTCCGAGAAATTCTCGCAGAGCTTACAGGA
CGAAAAGGAGGTTGTGCTAAAGGGAAAGGAGGATCGATGCACATGTATGCCAAGAACTTC
TACGGGGGCAATGGCATCGTGGGAGCGCAGGTGCCCCTGGGCGCTGGGATTGCTCTAGCC
TGTAAGTATAATGGAAAAGATGAGGTCTGCCTGACTTTATATGGCGATGGTGCTGCTAAC
CAGGGCCAGATATTCGAAGCTTACAACATGGCAGCTTTGTGGAAATTACCTTGTATTTTC
ATCTGTGAGAATAATCGCTATGGAATGGGAACGTCTGTTGAGAGAGCGGCAGCCAGCACT
GATTACTACAAGAGAGGCGATTTCATTCCTGGGCTGAGAGTGGATGGAATGGATATCCTG
TGCGTCCGAGAGGCAACAAGGTTTGCTGCTGCCTATTGTAGATCTGGGAAGGGGCCCATC
CTGATGGAGCTGCAGACTTACCGTTACCACGGACACAGTATGAGTGACCCTGGAGTCAGT
TACCGTACACGAGAAGAAATTCAGGAAGTAAGAAGTAAGAGTGACCCTATTATGCTTCTC
AAGGACAGGATGGTGAACAGCAATCTTGCCAGTGTGGAAGAACTAAAGGAAATTGATGTG
GAAGTGAGGAAGGAGATTGAGGATGCTGCCCAGTTTGCCACGGCCGATCCTGAGCCACCT
TTGGAAGAGCTGGGCTACCACATCTACTCCAGCGACCCACCTTTTGAAGTTCGTGGTGCC
AATCAGTGGATCAAGTTTAAGTCAGTCAGTTAA
GenBank Gene IDM27257
GeneCard IDNot Available
GenAtlas IDPDHA1
HGNC IDHGNC:8806
Chromosome LocationX
LocusXp22.2-p22.1
References
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