Tmic
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NamePyruvate dehydrogenase protein X component, mitochondrial
Synonyms
  • Dihydrolipoamide 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 pINot Available
GO Classification
Functions
  • transferase activity, transferring acyl groups
Processes
  • small molecule metabolic process
  • cellular metabolic process
  • pyruvate metabolic process
  • regulation of acetyl-CoA biosynthetic process from pyruvate
  • mitochondrial 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.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein IDNot Available
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 IDNot Available
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:21350
Chromosome Location11
LocusNot Available
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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