We use cookies to enhance the usability of our website. If you continue, we'll assume that you are happy to receive all cookies. More information. Don't show this again.
The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), 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 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Mar 2010]
Phobius predicted secreted proteins Predicted intracellular proteins Citric acid cycle related proteins Protein evidence (Ezkurdia et al 2014)
Show all
GO:0008152 [metabolic process] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0055114 [oxidation-reduction process]
Predicted intracellular proteins Citric acid cycle related proteins Protein evidence (Ezkurdia et al 2014)
Show all
GO:0008152 [metabolic process] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0055114 [oxidation-reduction process]
Enzymes ENZYME proteins Oxidoreductases SPOCTOPUS predicted membrane proteins Phobius predicted secreted proteins Predicted intracellular proteins Citric acid cycle related proteins Disease related genes Potential drug targets Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004738 [pyruvate dehydrogenase activity] GO:0004739 [pyruvate dehydrogenase (acetyl-transferring) activity] GO:0005634 [nucleus] GO:0005739 [mitochondrion] GO:0005759 [mitochondrial matrix] GO:0006006 [glucose metabolic process] GO:0006086 [acetyl-CoA biosynthetic process from pyruvate] GO:0006090 [pyruvate metabolic process] GO:0006099 [tricarboxylic acid cycle] GO:0008152 [metabolic process] GO:0010510 [regulation of acetyl-CoA biosynthetic process from pyruvate] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0043231 [intracellular membrane-bounded organelle] GO:0044237 [cellular metabolic process] GO:0044281 [small molecule metabolic process] GO:0045254 [pyruvate dehydrogenase complex]
SPOCTOPUS predicted membrane proteins Phobius predicted secreted proteins Predicted intracellular proteins Citric acid cycle related proteins Protein evidence (Ezkurdia et al 2014)
Show all
GO:0008152 [metabolic process] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0055114 [oxidation-reduction process]
Predicted secreted proteins Secreted proteins predicted by MDSEC Phobius predicted secreted proteins SPOCTOPUS predicted secreted proteins Citric acid cycle related proteins Protein evidence (Ezkurdia et al 2014)
Show all
GO:0008152 [metabolic process] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0055114 [oxidation-reduction process]
Enzymes ENZYME proteins Oxidoreductases SPOCTOPUS predicted membrane proteins Predicted secreted proteins Secreted proteins predicted by MDSEC Phobius predicted secreted proteins SPOCTOPUS predicted secreted proteins Citric acid cycle related proteins Disease related genes Potential drug targets Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004738 [pyruvate dehydrogenase activity] GO:0004739 [pyruvate dehydrogenase (acetyl-transferring) activity] GO:0005634 [nucleus] GO:0005739 [mitochondrion] GO:0005759 [mitochondrial matrix] GO:0006006 [glucose metabolic process] GO:0006086 [acetyl-CoA biosynthetic process from pyruvate] GO:0006090 [pyruvate metabolic process] GO:0006099 [tricarboxylic acid cycle] GO:0008152 [metabolic process] GO:0010510 [regulation of acetyl-CoA biosynthetic process from pyruvate] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0034604 [pyruvate dehydrogenase (NAD+) activity] GO:0043209 [myelin sheath] GO:0043231 [intracellular membrane-bounded organelle] GO:0044237 [cellular metabolic process] GO:0044281 [small molecule metabolic process] GO:0045254 [pyruvate dehydrogenase complex] GO:0061732 [mitochondrial acetyl-CoA biosynthetic process from pyruvate]
Enzymes ENZYME proteins Oxidoreductases Phobius predicted secreted proteins Predicted intracellular proteins Citric acid cycle related proteins Disease related genes Potential drug targets Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004738 [pyruvate dehydrogenase activity] GO:0004739 [pyruvate dehydrogenase (acetyl-transferring) activity] GO:0005634 [nucleus] GO:0005739 [mitochondrion] GO:0005759 [mitochondrial matrix] GO:0006006 [glucose metabolic process] GO:0006086 [acetyl-CoA biosynthetic process from pyruvate] GO:0006090 [pyruvate metabolic process] GO:0006099 [tricarboxylic acid cycle] GO:0008152 [metabolic process] GO:0010510 [regulation of acetyl-CoA biosynthetic process from pyruvate] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0044237 [cellular metabolic process] GO:0044281 [small molecule metabolic process] GO:0045254 [pyruvate dehydrogenase complex]
Enzymes ENZYME proteins Oxidoreductases SPOCTOPUS predicted membrane proteins Phobius predicted secreted proteins Predicted intracellular proteins Citric acid cycle related proteins Disease related genes Potential drug targets Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004738 [pyruvate dehydrogenase activity] GO:0004739 [pyruvate dehydrogenase (acetyl-transferring) activity] GO:0005634 [nucleus] GO:0005739 [mitochondrion] GO:0005759 [mitochondrial matrix] GO:0006006 [glucose metabolic process] GO:0006086 [acetyl-CoA biosynthetic process from pyruvate] GO:0006090 [pyruvate metabolic process] GO:0006099 [tricarboxylic acid cycle] GO:0008152 [metabolic process] GO:0010510 [regulation of acetyl-CoA biosynthetic process from pyruvate] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0043231 [intracellular membrane-bounded organelle] GO:0044237 [cellular metabolic process] GO:0044281 [small molecule metabolic process] GO:0045254 [pyruvate dehydrogenase complex]
