Pyruvate dehydrogenase protein X component, mitochondrial

NamePyruvate dehydrogenase protein X component, mitochondrial
SynonymsDihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex E3-binding protein E3BP Lipoyl-containing pyruvate dehydrogenase complex component X PDX1 proX
Gene NamePDHX
OrganismHuman
Amino acid sequence
>lcl|BSEQ0008819|Pyruvate dehydrogenase protein X component, mitochondrial
MAASWRLGCDPRLLRYLVGFPGRRSVGLVKGALGWSVSRGANWRWFHSTQWLRGDPIKIL
MPSLSPTMEEGNIVKWLKKEGEAVSAGDALCEIETDKAVVTLDASDDGILAKIVVEEGSK
NIRLGSLIGLIVEEGEDWKHVEIPKDVGPPPPVSKPSEPRPSPEPQISIPVKKEHIPGTL
RFRLSPAARNILEKHSLDASQGTATGPRGIFTKEDALKLVQLKQTGKITESRPTPAPTAT
PTAPSPLQATAGPSYPRPVIPPVSTPGQPNAVGTFTEIPASNIRRVIAKRLTESKSTVPH
AYATADCDLGAVLKVRQDLVKDDIKVSVNDFIIKAAAVTLKQMPDVNVSWDGEGPKQLPF
IDISVAVATDKGLLTPIIKDAAAKGIQEIADSVKALSKKARDGKLLPEEYQGGSFSISNL
GMFGIDEFTAVINPPQACILAVGRFRPVLKLTEDEEGNAKLQQRQLITVTMSSDSRVVDD
ELATRFLKSFKANLENPIRLA
Number of residues501
Molecular Weight54121.76
Theoretical pINone
GO Classification
Functions
    transferase activity, transferring acyl groups
Processes
    mitochondrial acetyl-CoA biosynthetic process from pyruvate
small molecule metabolic process
cellular metabolic process
pyruvate metabolic process
regulation of acetyl-CoA biosynthetic process from pyruvate
Components
    pyruvate dehydrogenase complex
mitochondrial matrix
General FunctionTransferase activity, transferring acyl groups
Specific FunctionRequired for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
Transmembrane Regions
GenBank Protein ID
UniProtKB IDO00330
UniProtKB Entry NameODPX_HUMAN
Cellular LocationMitochondrion matrix
Gene sequence
>lcl|BSEQ0020820|Pyruvate dehydrogenase protein X component, mitochondrial (PDHX)
ATGCAATCAGGCGGCGCTGAGGGCAGCCCGGGGGCGGGGCGAACGGGGCGGGGGCCGGGG
TCTGGTAAGGCCCCGCCGGCTGAGATATCCAGCGGCGCACCTGACTTCCCGGGAGGTGAT
CCCATTAAGATACTAATGCCATCACTGTCTCCTACAATGGAAGAAGGAAACATTGTGAAA
TGGCTGAAAAAGGAAGGTGAAGCGGTGAGTGCTGGAGATGCATTATGTGAAATTGAGACT
GACAAAGCTGTGGTTACCTTAGATGCAAGTGATGATGGAATCTTGGCCAAAATCGTGGTT
GAAGAAGGAAGTAAAAATATACGGCTAGGTTCACTAATTGGTTTGATAGTAGAAGAAGGA
GAAGATTGGAAACATGTTGAAATTCCCAAAGACGTAGGTCCTCCACCACCAGTTTCAAAA
CCTTCAGAGCCTCGCCCCTCACCAGAACCACAGATTTCCATCCCTGTCAAGAAGGAACAC
ATACCCGGGACACTACGGTTCCGTTTAAGTCCAGCTGCCCGCAATATTCTGGAAAAACAC
TCACTGGATGCTAGCCAGGGCACAGCCACTGGCCCTCGGGGGATATTCACTAAAGAGGAT
GCTCTCAAACTTGTCCAGTTGAAACAAACGGGCAAGATTACCGAGTCCAGACCAACTCCA
GCCCCCACAGCCACTCCCACAGCACCTTCGCCCCTACAGGCCACAGCTGGACCATCTTAT
CCCCGGCCTGTGATCCCACCAGTATCAACTCCTGGACAACCCAATGCAGTGGGCACATTC
ACTGAAATCCCCGCCAGCAATATTCGAAGAGTTATTGCCAAGAGATTAACTGAATCTAAA
AGTACTGTACCTCATGCATATGCTACTGCTGACTGTGACCTTGGAGCTGTTTTAAAAGTT
AGGCAAGATCTGGTCAAAGATGACATTAAAGTATCAGTAAATGATTTTATCATCAAGGCA
GCAGCTGTTACCCTTAAACAAATGCCAGATGTTAATGTAAGCTGGGATGGAGAGGGCCCA
AAGCAACTGCCATTTATTGACATTTCAGTGGCTGTGGCAACAGATAAAGGCTTACTTACT
CCAATCATAAAAGATGCTGCTGCTAAAGGTATCCAGGAAATTGCTGACTCTGTAAAGGCT
CTATCAAAGAAAGCAAGAGATGGAAAATTGTTGCCTGAAGAATACCAAGGAGGATCTTTT
AGTATTTCCAACTTGGGGATGTTTGGCATCGACGAATTTACTGCAGTGATTAACCCTCCT
CAGGCCTGCATTTTGGCGGTTGGGAGGTTCCGACCTGTGCTGAAGCTCACTGAGGATGAA
GAGGGAAATGCCAAACTGCAGCAGCGCCAGCTCATAACAGTCACAATGTCAAGTGACAGT
CGAGTGGTTGATGACGAACTGGCAACCAGGTTTCTTAAAAGTTTTAAAGCAAACCTAGAG
AATCCTATCCGACTTGCCTAG
GenBank Gene ID
GeneCard IDNone
GenAtlas ID
HGNC IDHGNC:21350
Chromosome Location11
LocusNone
References
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  2. Aral B, Benelli C, Ait-Ghezala G, Amessou M, Fouque F, Maunoury C, Creau N, Kamoun P, Marsac C: 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. 1997 Dec;61(6):1318-26.[9399911 ]
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  7. Hiromasa Y, Fujisawa T, Aso Y, Roche TE: 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. 2004 Feb 20;279(8):6921-33. Epub 2003 Nov 24.[14638692 ]
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  11. Vaca Jacome AS, Rabilloud T, Schaeffer-Reiss C, Rompais M, Ayoub D, Lane L, Bairoch A, Van Dorsselaer A, Carapito C: N-terminome analysis of the human mitochondrial proteome. Proteomics. 2015 Jul;15(14):2519-24. doi: 10.1002/pmic.201400617. Epub 2015 Jun 8.[25944712 ]
  12. Ciszak EM, Makal A, Hong YS, Vettaikkorumakankauv AK, Korotchkina LG, Patel MS: How dihydrolipoamide dehydrogenase-binding protein binds dihydrolipoamide dehydrogenase in the human pyruvate dehydrogenase complex. J Biol Chem. 2006 Jan 6;281(1):648-55. Epub 2005 Nov 1.[16263718 ]
  13. Brautigam CA, Wynn RM, Chuang JL, Machius M, Tomchick DR, Chuang DT: Structural insight into interactions between dihydrolipoamide dehydrogenase (E3) and E3 binding protein of human pyruvate dehydrogenase complex. Structure. 2006 Mar;14(3):611-21. Epub 2006 Jan 26.[16442803 ]
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