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PDHA1 pyruvate dehydrogenase (lipoamide) alpha 1 [Homo sapiens (human)]


RefSeq Accession Definition Service Stock Status Price *Turnaround time Order
NM_000284 Homo sapiens pyruvate dehydrogenase (lipoamide) alpha 1 (PDHA1), transcript variant 1, mRNA. GenEZ ORF Cloning In-stock $418.00 $369.00 10
NM_001173454 Homo sapiens pyruvate dehydrogenase (lipoamide) alpha 1 (PDHA1), transcript variant 2, mRNA. GenEZ ORF Cloning On-demand $699.00 18
NM_001173455 Homo sapiens pyruvate dehydrogenase (lipoamide) alpha 1 (PDHA1), transcript variant 3, mRNA. GenEZ ORF Cloning On-demand $549.00 14
NM_001173456 Homo sapiens pyruvate dehydrogenase (lipoamide) alpha 1 (PDHA1), transcript variant 4, mRNA. GenEZ ORF Cloning On-demand $549.00 14
XM_006724495 PREDICTED: Homo sapiens pyruvate dehydrogenase (lipoamide) alpha 1 (PDHA1), transcript variant X1, mRNA. GenEZ ORF Cloning On-demand $549.00 14

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Gene Symbol PDHA1
Entrez Gene ID 5160
Full Name pyruvate dehydrogenase (lipoamide) alpha 1
Synonyms PDHA, PDHCE1A, PHE1A
General protein information
Preferred Names
pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
Names
pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
PDHE1-A type I
pyruvate dehydrogenase complex, E1-alpha polypeptide 1
NP_000275.1
NP_001166925.1
NP_001166926.1
NP_001166927.1
Gene Type protein-coding
Organism Homo sapiens (human)
Genome

X

Xp22.1

Summary 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].
Disorder

MIM: 300502

Pyruvate dehydrogenase deficiency, 312170 (3); Leigh syndrome,

mRNA Protein Product Sequence Price Select
NM_000284, 291084749 NP_000275, 4505685 pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial isoform 1 precursor ORF Sequence $269.00
NM_001173454, 291084741 NP_001166925, 291084742 pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial isoform 2 precursor ORF Sequence $550.00
NM_001173455, 291084743 NP_001166926, 291084744 pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial isoform 3 precursor ORF Sequence $400.00
NM_001173456, 291084756 NP_001166927, 291084757 pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial isoform 4 precursor ORF Sequence $400.00
XM_006724495, 578837911 XP_006724558, 578837912 pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial isoform X1 ORF Sequence $400.00
KEGG
hsa00020Citrate cycle (TCA cycle)
hsa00010Glycolysis / Gluconeogenesis
hsa00620Pyruvate metabolism
hsa_M00307Pyruvate oxidation, pyruvate => acetyl-CoA
hsa04066HIF-1 signaling pathway
hsa01200Carbon metabolism
WikiPathways
WP534Glycolysis and Gluconeogenesis
WP528Acetylcholine Synthesis
BIOCYC
META_PYRUVDEHYD-PWYpyruvate decarboxylation to acetyl CoA
HUMAN_PYRUVDEHYD-PWYpyruvate decarboxylation to acetyl CoA
Reactome
REACT_12528Regulation of pyruvate dehydrogenase (PDH) complex
REACT_2071Pyruvate metabolism
REACT_1046Pyruvate metabolism and Citric Acid (TCA) cycle
REACT_111217Metabolism
REACT_111083The citric acid (TCA) cycle and respiratory electron transport
Homo sapiens (human)PDHA1NP_001166926.1
Pan troglodytes (chimpanzee)PDHA1NP_001104283.1
Macaca mulatta (Rhesus monkey)LOC719277XP_001113273.2
Canis lupus familiaris (dog)PDHA1XP_003435519.1
Bos taurus (cattle)PDHA1NP_001094516.1
Mus musculus (house mouse)Pdha1NP_032836.1
Rattus norvegicus (Norway rat)Pdha1NP_001004072.2
Gallus gallus (chicken)PDHA1NP_001012562.1
Danio rerio (zebrafish)pdha1bNP_001002399.1
Danio rerio (zebrafish)pdha1aNP_998558.1
Caenorhabditis eleganspdha-1NP_871953.1
Arabidopsis thaliana (thale cress)IAR4NP_173828.1
Arabidopsis thaliana (thale cress)E1 ALPHANP_176198.1
Xenopus (Silurana) tropicalis (western clawed frog)pdha1NP_001017072.1
Process
IDNameEvidence
GO:0006006glucose metabolic processIEA
GO:0006086acetyl-CoA biosynthetic process from pyruvateIDA
GO:0006090pyruvate metabolic processTAS
GO:0006096glycolytic processIEA
GO:0006099tricarboxylic acid cycleIDA
GO:0010510regulation of acetyl-CoA biosynthetic process from pyruvateTAS
GO:0044237cellular metabolic processTAS
GO:0044281small molecule metabolic processTAS
Component
IDNameEvidence
GO:0005634nucleusIDA
GO:0005739mitochondrionIDA
GO:0005759mitochondrial matrixTAS
GO:0045254pyruvate dehydrogenase complexIDA
Function
IDNameEvidence
GO:0004738pyruvate dehydrogenase activityIDA
GO:0004739pyruvate dehydrogenase (acetyl-transferring) activityIEA
GeneCards PDHA1
UniProt P08559
Vega OTTHUMG00000021224
MIM 300502
Ensembl ENSG00000131828
HGNC 8806
HPRD 02420

GeneRIFs: Gene References Into Functions What's a GeneRIF?

What is the normal function of the PDHA1 gene?

The PDHA1 gene provides instructions for making a protein called E1 alpha. The E1 alpha protein is a piece (a subunit) of a larger protein: two E1 alpha proteins combine with two copies of another protein called E1 beta (produced from the PDHB gene) to form the E1 enzyme. This enzyme, also known as pyruvate dehydrogenase, is a component of a group of proteins called the pyruvate dehydrogenase complex.

The pyruvate dehydrogenase complex plays an important role in the pathways that convert the energy from food into a form that cells can use. This complex converts a molecule called pyruvate, which is formed from the breakdown of carbohydrates, into another molecule called acetyl-CoA. The E1 enzyme performs one part of this chemical reaction. The conversion of pyruvate is essential to begin the series of chemical reactions that produces adenosine triphosphate (ATP), the cell's main energy source.

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