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Identification
HMDB Protein ID HMDBP00041
Secondary Accession Numbers
  • 5270
Name Pyruvate dehydrogenase protein X component, mitochondrial
Synonyms
  1. Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex
  2. E3-binding protein
  3. E3BP
  4. Lipoyl-containing pyruvate dehydrogenase complex component X
  5. proX
Gene Name PDHX
Protein Type Enzyme
Biological Properties
General Function Involved in acyltransferase activity
Specific Function Required 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
Pathways Not Available
Reactions Not Available
GO Classification
Function
binding
catalytic activity
transferase activity
transferase activity, transferring acyl groups
transferase activity, transferring acyl groups other than amino-acyl groups
acyltransferase activity
protein binding
Process
metabolic process
Cellular Location
  1. Mitochondrion matrix
Gene Properties
Chromosome Location Chromosome:1
Locus 11p13
SNPs PDHX
Gene Sequence
>1506 bp
ATGGCGGCCTCCTGGAGGCTGGGCTGTGATCCGCGGCTGCTGCGTTATCTTGTGGGCTTC
CCTGGCTGCCGAAGCGTAGGGCTGGTGAAGGGGGCTCTTGGGTGGTCCGTAAGCCGCGGA
GCTAATTGGAGATGGTTTCACAGCACGCAGTGGCTTCGGGGTGATCCCATTAAGATACTA
ATGCCATCACTGTCTCCTACAATGGAAGAAGGAAACATTGTGAAATGGCTGAAAAAGGAA
GGTGAAGCGGTGAGTGCTGGAGATGCATTATGTGAAATTGAGACTGACAAAGCTGTGGTT
ACCTTAGATGCAAGTGATGATGGAATCTTGGCCAAAATCGTGGTTGAAGAAGGAAGTAAA
AATATACGGCTAGGTTCACTAATTGGTTTGATAGTAGAAGAAGGAGAAGATTGGAAACAT
GTTGAAATTCCCAAAGACGTAGGTCCTCCACCACCAGTTTCAAAACCTTCAGAGCCTCGC
CCCTCACCAGAACCACAGATTTCCATCCCTGTCAAGAAGGAACACATACCCGGGACACTA
CGGTTCCGTTTAAGTCCAGCTGCCCGCAATATTCTGGAAAAACACTCACTGGATGCTAGC
CAGGGCACAGCCACTGGCCCTCGGGGGATATTCACTAAAGAGGATGCTCTCAAACTTGTC
CAGTTGAAACAAACGGGCAAGATTACCGAGTCCAGACCAACTCCAGCCCCCACAGCCACT
CCCACAGCACCTTCGCCCCTACAGGCCACATCTGGACCATCTTATCCCCGGCCTGTGATC
CCACCAGTATCAACTCCCGGACAACCCAATGCAGTGGGCACATTCACTGAAATCCCCGCC
AGCAATATTCGAAGAGTTATTGCCAAGAGATTAACTGAATCTAAAAGTACTGTACCTCAT
GCATATGCTACTGCTGACTGTGACCTTGGAGCTGTTTTAAAAGTTAGGCAAGATCTGGTC
AAAGATGACATTAAAGTATCAGTAAATGATTTTATCATCAAGGCAGCAGCTGTTACCCTT
AAACAAATGCCAGATGTTAATGTAAGCTGGGATGGAGAGGGCCCAAAGCAACTGCCATTT
ATTGACATTTCAGTGGCTGTGGCAACAGATAAAGGCTTACTTACTCCAATCATAAAAGAT
GCTGCTGCTAAAGGTATCCAGGAAATTGCTGACTCTGTAAAGGCTCTATCAAAGAAAGCA
AGAGATGGAAAATTGTTGCCTGAAGAATACCAAGGAGGATCTTTTAGTATTTCCAACTTG
GGGATGTTTGGCATCGACGAATTTACTGCAGTGATTAACCCTCCTCAGGCCTGCATTTTG
GCGGTTGGGAGGTTCCGACCTGTGCTGAAGCTCACTGAGGATGAAGAGGGAAATGCCAAA
CTGCAGCAGCGCCAGCTCATAACAGTCACAATGTCAAGTGACAGTCGAGTGGTTGATGAC
GAACTGGCAACCAGGTTTCTTAAAAGTTTTAAAGCAAACCTAGAGAATCCTATCCGACTT
GCCTAG
Protein Properties
Number of Residues 501
Molecular Weight 54121.8
Theoretical pI 9.09
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • None
Protein Sequence
>Pyruvate dehydrogenase protein X component, mitochondrial
MAASWRLGCDPRLLRYLVGFPGRRSVGLVKGALGWSVSRGANWRWFHSTQWLRGDPIKIL
MPSLSPTMEEGNIVKWLKKEGEAVSAGDALCEIETDKAVVTLDASDDGILAKIVVEEGSK
NIRLGSLIGLIVEEGEDWKHVEIPKDVGPPPPVSKPSEPRPSPEPQISIPVKKEHIPGTL
RFRLSPAARNILEKHSLDASQGTATGPRGIFTKEDALKLVQLKQTGKITESRPTPAPTAT
PTAPSPLQATAGPSYPRPVIPPVSTPGQPNAVGTFTEIPASNIRRVIAKRLTESKSTVPH
AYATADCDLGAVLKVRQDLVKDDIKVSVNDFIIKAAAVTLKQMPDVNVSWDGEGPKQLPF
IDISVAVATDKGLLTPIIKDAAAKGIQEIADSVKALSKKARDGKLLPEEYQGGSFSISNL
GMFGIDEFTAVINPPQACILAVGRFRPVLKLTEDEEGNAKLQQRQLITVTMSSDSRVVDD
ELATRFLKSFKANLENPIRLA
GenBank ID Protein 2316040
UniProtKB/Swiss-Prot ID O00330
UniProtKB/Swiss-Prot Entry Name ODPX_HUMAN
PDB IDs Not Available
GenBank Gene ID AF001437
GeneCard ID PDHX
GenAtlas ID PDHX
HGNC ID HGNC:21350
References
General References
  1. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  2. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [PubMed:19608861 ]
  3. Harris RA, Bowker-Kinley MM, Wu P, Jeng J, Popov KM: 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. 1997 Aug 8;272(32):19746-51. [PubMed:9242632 ]
  4. 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. [PubMed:9399911 ]
  5. Ling M, McEachern G, Seyda A, MacKay N, Scherer SW, Bratinova S, Beatty B, Giovannucci-Uzielli ML, Robinson BH: Detection of a homozygous four base pair deletion in the protein X gene in a case of pyruvate dehydrogenase complex deficiency. Hum Mol Genet. 1998 Mar;7(3):501-5. [PubMed:9467010 ]
  6. Yu W, Andersson B, Worley KC, Muzny DM, Ding Y, Liu W, Ricafrente JY, Wentland MA, Lennon G, Gibbs RA: Large-scale concatenation cDNA sequencing. Genome Res. 1997 Apr;7(4):353-8. [PubMed:9110174 ]
  7. 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. [PubMed:16263718 ]
  8. 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. [PubMed:16442803 ]