Pyruvate Dehydrogenase Complex Component X (PDHX)

E3BP; proX; PDX1; OPDX; DLDBP; E3-binding protein; Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex

SPN	P309
HGNC	21350
MGI	1351627
PubMed	21194677
RGD	1566332
UniProt	O00330

The PDHX gene encodes component X of the pyruvate dehydrogenase (PDH) complex. For a detailed description of the pyruvate dehydrogenase complex. The mammalian PDH complex differs from that in E. coli and from the other mammalian alpha-keto acid dehydrogenases by the presence of a 53-kD protein called protein X. Component X binds to the E3 component of the PDH complex.

The open reading frame encodes an 501-amino acid protein sequence that is 20% identical with that encoded by the yeast PDX1 gene and 40% identical with that encoded by the lipoacyltransferase component E2 of the PDH (E2), and includes a lipoyl-bearing domain. Sequence comparisons showed that the PDHX protein contains an E3-binding domain.

Reference

"Dihydrolipoamide dehydrogenase-binding protein of the human pyruvate dehydrogenase complex. DNA-derived amino acid sequence, expression, and reconstitution of the pyruvate dehydrogenase complex."
J. Biol. Chem. 272:19746-19751(1997) [PubMed] [Europe PMC] [Abstract]

"Mutations in PDX1, the human lipoyl-containing component X of the pyruvate dehydrogenase-complex gene on chromosome 11p1, in congenital lactic acidosis."
Am. J. Hum. Genet. 61:1318-1326(1997) [PubMed] [Europe PMC] [Abstract]

"Detection of a homozygous four base pair deletion in the protein X gene in a case of pyruvate dehydrogenase complex deficiency."
Hum. Mol. Genet. 7:501-505(1998) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"Human chromosome 11 DNA sequence and analysis including novel gene identification."
Nature 440:497-500(2006) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Large-scale concatenation cDNA sequencing."
Genome Res. 7:353-358(1997) [PubMed] [Europe PMC] [Abstract]

"Organization of the cores of the mammalian pyruvate dehydrogenase complex formed by E2 and E2 plus the E3-binding protein and their capacities to bind the E1 and E3 components."
J. Biol. Chem. 279:6921-6933(2004) [PubMed] [Europe PMC] [Abstract]

"Lysine acetylation targets protein complexes and co-regulates major cellular functions."
Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]

"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"How dihydrolipoamide dehydrogenase-binding protein binds dihydrolipoamide dehydrogenase in the human pyruvate dehydrogenase complex."
J. Biol. Chem. 281:648-655(2006) [PubMed] [Europe PMC] [Abstract]

"Structural insight into interactions between dihydrolipoamide dehydrogenase (E3) and E3 binding protein of human pyruvate dehydrogenase complex."
Structure 14:611-621(2006) [PubMed] [Europe PMC] [Abstract]

"Solution structure of RSGI RUH-054, a lipoyl domain from human 2-oxoacid dehydrogenase."
Submitted (OCT-2006) to the PDB data bank