Mus musculus Gene: Pdha1
Summary
InnateDB Gene IDBG-182646.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol Pdha1
Gene Name pyruvate dehydrogenase E1 alpha 1
Synonyms Pdha-1;
Species Mus musculus
Ensembl Gene ENSMUSG00000031299
Encoded Proteins
pyruvate dehydrogenase E1 alpha 1
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary This gene does not have any Entrez summary - the following is the summary from its human ortholog ENSG00000131828:
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]
Gene Information
Type Protein coding
Genomic Location Chromosome X:160122209-160138413
Strand Reverse strand
Band F4
Transcripts
ENSMUST00000033662 ENSMUSP00000033662
ENSMUST00000156531
ENSMUST00000126440
ENSMUST00000127535
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 11 experimentally validated interaction(s) in this database.
They are also associated with 22 interaction(s) predicted by orthology.
Experimentally validated
Total 11 [view]
Protein-Protein 11 [view]
Protein-DNA 0
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Predicted by orthology
Total 22 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0004738 pyruvate dehydrogenase activity
GO:0004739 pyruvate dehydrogenase (acetyl-transferring) activity
GO:0016624 oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
Biological Process
GO:0006006 glucose metabolic process
GO:0006086 acetyl-CoA biosynthetic process from pyruvate
GO:0006096 glycolytic process
GO:0006099 tricarboxylic acid cycle
GO:0008152 metabolic process
GO:0055114 oxidation-reduction process
Cellular Component
GO:0005634 nucleus
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
GO:0043231 intracellular membrane-bounded organelle
GO:0045254 pyruvate dehydrogenase complex
Orthologs
Species
Homo sapiens
Bos taurus
Gene ID
Gene Order
Method
Confidence
Comments
SSD Ortholog
Ortholog supports species divergence
Not yet available
SSD Ortholog
Ortholog supports species divergence
Pathways
NETPATH
REACTOME
The citric acid (TCA) cycle and respiratory electron transport pathway
Regulation of pyruvate dehydrogenase (PDH) complex pathway
Metabolism pathway
Pyruvate metabolism pathway
Pyruvate metabolism and Citric Acid (TCA) cycle pathway
KEGG
Glycolysis / Gluconeogenesis pathway
Citrate cycle (TCA cycle) pathway
Pyruvate metabolism pathway
INOH
PID BIOCARTA
PID NCI
Pathway Predictions based on Human Orthology Data
NETPATH
REACTOME
Pyruvate metabolism and Citric Acid (TCA) cycle pathway
Pyruvate metabolism pathway
The citric acid (TCA) cycle and respiratory electron transport pathway
Metabolism pathway
Regulation of pyruvate dehydrogenase (PDH) complex pathway
Regulation of pyruvate dehydrogenase (PDH) complex pathway
The citric acid (TCA) cycle and respiratory electron transport pathway
Pyruvate metabolism pathway
Metabolism pathway
Pyruvate metabolism and Citric Acid (TCA) cycle pathway
KEGG
Glycolysis / Gluconeogenesis pathway
Citrate cycle (TCA cycle) pathway
Pyruvate metabolism pathway
INOH
Pyruvate metabolism pathway
PID BIOCARTA
PID NCI
Cross-References
SwissProt P35486
TrEMBL Q3UFJ3
UniProt Splice Variant
Entrez Gene 18597
UniGene Mm.34775
RefSeq NM_008810
OMIM
CCDS CCDS41195
HPRD
IMGT
MGI ID MGI:97532
MGI Symbol Pdha1
EMBL AK145718 AK148459 AK167987 AK168152 AK169289 BC007142 CH466571 M76727
GenPept AAA53046 AAH07142 BAE26608 BAE28567 BAE39978 BAE40117 BAE41046 EDL40808
RNA Seq Atlas 18597