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PDHB pyruvate dehydrogenase (lipoamide) beta [Homo sapiens (human)]


RefSeq Accession Definition Service Stock Status Price *Turnaround time Order
NM_000925 Homo sapiens pyruvate dehydrogenase (lipoamide) beta (PDHB), transcript variant 1, mRNA. GenEZ ORF Cloning In-stock $418.00 $369.00 10
NM_001173468 Homo sapiens pyruvate dehydrogenase (lipoamide) beta (PDHB), transcript variant 2, mRNA. GenEZ ORF Cloning On-demand $549.00 14

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Gene Symbol PDHB
Entrez Gene ID 5162
Full Name pyruvate dehydrogenase (lipoamide) beta
Synonyms PDHBD, PDHE1-B, PHE1B
General protein information
Preferred Names
pyruvate dehydrogenase E1 component subunit beta, mitochondrial
Names
pyruvate dehydrogenase E1 component subunit beta, mitochondrial
pyruvate dehydrogenase, E1 beta polypeptide
NP_000916.2
NP_001166939.1
Gene Type protein-coding
Organism Homo sapiens (human)
Genome

3

3p21.1-p14.2

Summary The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and carbon dioxide, 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 beta subunit. Mutations in this gene are associated with pyruvate dehydrogenase E1-beta deficiency. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Mar 2012].
Disorder

MIM: 179060

Pyruvate dehydrogenase E1-beta deficiency (3)

mRNA Protein Product Sequence Price Select
NM_000925, 291084856 NP_000916, 156564403 pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 1 precursor ORF Sequence $269.00
NM_001173468, 291084857 NP_001166939, 291084858 pyruvate dehydrogenase E1 component subunit beta, mitochondrial isoform 2 precursor 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
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)PDHBNP_000916.2
Pan troglodytes (chimpanzee)PDHBNP_001233347.1
Macaca mulatta (Rhesus monkey)PDHBXP_001096445.1
Canis lupus familiaris (dog)PDHBXP_533778.2
Bos taurus (cattle)PDHBNP_001030512.2
Mus musculus (house mouse)PdhbNP_077183.1
Rattus norvegicus (Norway rat)PdhbNP_001007621.1
Gallus gallus (chicken)PDHBNP_001185549.1
Danio rerio (zebrafish)pdhbNP_998319.1
Drosophila melanogaster (fruit fly)CG11876NP_651668.1
Caenorhabditis eleganspdhb-1NP_500340.1
Arabidopsis thaliana (thale cress)MAB1NP_199898.1
Xenopus (Silurana) tropicalis (western clawed frog)pdhbNP_001016212.1
Process
IDNameEvidence
GO:0006006glucose metabolic processIEA
GO:0006086acetyl-CoA biosynthetic process from pyruvateIDA
GO:0006090pyruvate metabolic processTAS
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:0005759mitochondrial matrixTAS
GO:0045254pyruvate dehydrogenase complexIDA
GO:0070062extracellular vesicular exosomeIDA
Function
IDNameEvidence
GO:0004738pyruvate dehydrogenase activityIDA
GO:0004739pyruvate dehydrogenase (acetyl-transferring) activityIEA
GO:0005515protein bindingIPI
GeneCards PDHB
UniProt P11177
Vega OTTHUMG00000159157
MIM 179060
Ensembl ENSG00000168291
HGNC 8808
HPRD 01530

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

What is the normal function of the PDHB gene?

The PDHB gene provides instructions for making a protein called E1 beta. E1 beta is a piece (a subunit) of a larger protein: two E1 beta proteins combine with two copies of another protein, called E1 alpha (produced from the PDHA1 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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