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Pyruvate dehydrogenase (lipoamide) beta

PDHB
PDB 3exi EBI.png
Available structures
PDB Ortholog search: PDBe RCSB
Identifiers
Aliases PDHB, PDHBD, PDHE1-B, PHE1B, Pyruvate dehydrogenase (lipoamide) beta, PDHE1B, pyruvate dehydrogenase E1 beta subunit
External IDs MGI: 1915513 HomoloGene: 712 GeneCards: PDHB
Gene location (Human)
Chromosome 3 (human)
Chr. Chromosome 3 (human)
Chromosome 3 (human)
Genomic location for PDHB
Genomic location for PDHB
Band 3p14.3 Start 58,427,630 bp
End 58,433,857 bp
Orthologs
Species Human Mouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_000925
NM_001173468
NM_001315536

NM_024221

RefSeq (protein)

NP_000916
NP_001166939
NP_001302465

NP_077183

Location (UCSC) Chr 3: 58.43 – 58.43 Mb Chr 3: 8.17 – 8.17 Mb
PubMed search

1NI4, 2OZL, 3EXE, 3EXF, 3EXG, 3EXH, 3EXI

NM_000925
NM_001173468
NM_001315536

NM_024221

NP_000916
NP_001166939
NP_001302465

NP_077183

Pyruvate dehydrogenase (lipoamide) beta, also known as pyruvate dehydrogenase E1 component subunit beta, mitochondrial or PDHE1-B is an enzyme that in humans is encoded by the PDHB gene. The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2, 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.


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