Sequence of ODPA_HUMAN
EC Number:1.2.4.1
EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
Reaction
pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO2
Other sequences found for EC No. 1.2.4.1
General information:
Sequence
0 MRKMLAAVSR VLSGASQKPA SRVLVASRNF ANDATFEIKK CDLHRLEEGP PVTTVLTRED
60 GLKYYRMMQT VRRMELKADQ LYKQKIIRGF CHLCDGQEAC CVGLEAGINP TDHLITAYRA
120 HGFTFTRGLS VREILAELTG RKGGCAKGKG GSMHMYAKNF YGGNGIVGAQ VPLGAGIALA
180 CKYNGKDEVC LTLYGDGAAN QGQIFEAYNM AALWKLPCIF ICENNRYGMG TSVERAAAST
240 DYYKRGDFIP GLRVDGMDIL CVREATRFAA AYCRSGKGPI LMELQTYRYH GHSMSDPGVS
300 YRTREEIQEV RSKSDPIMLL KDRMVNSNLA SVEELKEIDV EVRKEIEDAA QFATADPEPP
360 LEELGYHIYS SDPPFEVRGA NQWIKFKSVS
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Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
580253
Koike K.,Urata Y.,Matsuo S.,Koike M.
Characterization and nucleotide sequence of the gene encoding the human pyruvate dehydrogenase alpha-subunit.
Gene
93
307-311
1990
580254
Ho L.,Wexler I.D.,Liu T.C.,Thekkumkara T.J.,Patel M.S.
Characterization of cDNAs encoding human pyruvate dehydrogenase alpha subunit.
Proc. Natl. Acad. Sci. U.S.A.
86
5330-5334
1989
580256
Dahl H.-H.M.,Hunt S.M.,Hutchison W.M.,Brown G.K.
The human pyruvate dehydrogenase complex. Isolation of cDNA clones for the E1 alpha subunit, sequence analysis, and characterization of the mRNA.
J. Biol. Chem.
262
7398-7403
1987
580257
Maragos C.,Hutchinson W.M.,Hayasaki K.,Brown G.K.,Dahl H.-H.M.
Structural organization of the gene for the E1 alpha subunit of the human pyruvate dehydrogenase complex.
J. Biol. Chem.
264
12294-12298
1989
580258
de Meirleir L.,MacKay N.,Wah A.M.L.H.,Robinson B.H.
Isolation of a full-length complementary DNA coding for human E1 alpha subunit of the pyruvate dehydrogenase complex.
J. Biol. Chem.
263
1991-1995
1988
580259
Koike K.,Ohta S.,Urata Y.,Kagawa Y.,Koike M.
Cloning and sequencing of cDNAs encoding alpha and beta subunits of human pyruvate dehydrogenase.
Proc. Natl. Acad. Sci. U.S.A.
85
41-45
1988
580261
Ota T.,Suzuki Y.,Nishikawa T.,Otsuki T.,Sugiyama T.,Irie R.,Wakamatsu A.,Hayashi K.,Sato H.,Nagai K.,Kimura K.,Makita H.,Sekine M.,Obayashi M.,Nishi T.,Shibahara T.,Tanaka T.,Ishii S.,Yamamoto J.,Saito K.,Kawai Y.,Isono Y.,Nakamura Y.,Nagahari K.,Murakami K.,Yasuda T.,Iwayanagi T.,Wagatsuma M.,Shiratori A.,Sudo H.,Hosoiri T.,Kaku Y.,Kodaira H.,Kondo H.,Sugawara M.,Takahashi M.,Kanda K.,Yokoi T.,Furuya T.,Kikkawa E.,Omura Y.,Abe K.,Kamihara K.,Katsuta N.,Sato K.,Tanikawa M.,Yamazaki M.,Ninomiya K.,Ishibashi T.,Yamashita H.,Murakawa K.,Fujimori K.,Tanai H.,Kimata M.,Watanabe M.,Hiraoka S.,Chiba Y.,Ishida S.,Ono Y.,Takiguchi S.,Watanabe S.,Yosida M.,Hotuta T.,Kusano J.,Kanehori K.,Takahashi-Fujii A.,Hara H.,Tanase T.-O.,Nomura Y.,Togiya S.,Komai F.,Hara R.,Takeuchi K.,Arita M.,Imose N.,Musashino K.,Yuuki H.,Oshima A.,Sasaki N.,Aotsuka S.,Yoshikawa Y.,Matsunawa H.,Ichihara T.,Shiohata N.,Sano S.,Moriya S.,Momiyama H.,Satoh N.,Takami S.,Terashima Y.,Suzuki O.,Nakagawa S.,Senoh A.,Mizoguchi H.,Goto Y.,Shimizu F.,Wakebe H.,Hishigaki H.,Watanabe T.,Sugiyama A.,Takemoto M.,Kawakami B.,Yamazaki M.,Watanabe K.,Kumagai A.,Itakura S.,Fukuzumi Y.,Fujimori Y.,Komiyama M.,Tashiro H.,Tanigami A.,Fujiwara T.,Ono T.,Yamada K.,Fujii Y.,Ozaki K.,Hirao M.,Ohmori Y.,Kawabata A.,Hikiji T.,Kobatake N.,Inagaki H.,Ikema Y.,Okamoto S.,Okitani R.,Kawakami T.,Noguchi S.,Itoh T.,Shigeta K.,Senba T.,Matsumura K.,Nakajima Y.,Mizuno T.,Morinaga M.,Sasaki M.,Togashi T.,Oyama M.,Hata H.,Watanabe M.,Komatsu T.,Mizushima-Sugano J.,Satoh T.,Shirai Y.,Takahashi Y.,Nakagawa K.,Okumura K.,Nagase T.,Nomura N.,Kikuchi H.,Masuho Y.,Yamashita R.,Nakai K.,Yada T.,Nakamura Y.,Ohara O.,Isogai T.,Sugano S.
Complete sequencing and characterization of 21,243 full-length human cDNAs.
Nat. Genet.
36
40-45
2004
580263
Ross M.T.,Grafham D.V.,Coffey A.J.,Scherer S.,McLay K.,Muzny D.,Platzer M.,Howell G.R.,Burrows C.,Bird C.P.,Frankish A.,Lovell F.L.,Howe K.L.,Ashurst J.L.,Fulton R.S.,Sudbrak R.,Wen G.,Jones M.C.,Hurles M.E.,Andrews T.D.,Scott C.E.,Searle S.,Ramser J.,Whittaker A.,Deadman R.,Carter N.P.,Hunt S.E.,Chen R.,Cree A.,Gunaratne P.,Havlak P.,Hodgson A.,Metzker M.L.,Richards S.,Scott G.,Steffen D.,Sodergren E.,Wheeler D.A.,Worley K.C.,Ainscough R.,Ambrose K.D.,Ansari-Lari M.A.,Aradhya S.,Ashwell R.I.,Babbage A.K.,Bagguley C.L.,Ballabio A.,Banerjee R.,Barker G.E.,Barlow K.F.,Barrett I.P.,Bates K.N.,Beare D.M.,Beasley H.,Beasley O.,Beck A.,Bethel G.,Blechschmidt K.,Brady N.,Bray-Allen S.,Bridgeman A.M.,Brown A.J.,Brown M.J.,Bonnin D.,Bruford E.A.,Buhay C.,Burch P.,Burford D.,Burgess J.,Burrill W.,Burton J.,Bye J.M.,Carder C.,Carrel L.,Chako J.,Chapman J.C.,Chavez D.,Chen E.,Chen G.,Chen Y.,Chen Z.,Chinault C.,Ciccodicola A.,Clark S.Y.,Clarke G.,Clee C.M.,Clegg S.,Clerc-Blankenburg K.,Clifford K.,Cobley V.,Cole C.G.,Conquer J.S.,Corby N.,Connor R.E.,David R.,Davies J.,Davis C.,Davis J.,Delgado O.,Deshazo D.,Dhami P.,Ding Y.,Dinh H.,Dodsworth S.,Draper H.,Dugan-Rocha S.,Dunham A.,Dunn M.,Durbin K.J.,Dutta I.,Eades T.,Ellwood M.,Emery-Cohen A.,Errington H.,Evans K.L.,Faulkner L.,Francis F.,Frankland J.,Fraser A.E.,Galgoczy P.,Gilbert J.,Gill R.,Gloeckner G.,Gregory S.G.,Gribble S.,Griffiths C.,Grocock R.,Gu Y.,Gwilliam R.,Hamilton C.,Hart E.A.,Hawes A.,Heath P.D.,Heitmann K.,Hennig S.,Hernandez J.,Hinzmann B.,Ho S.,Hoffs M.,Howden P.J.,Huckle E.J.,Hume J.,Hunt P.J.,Hunt A.R.,Isherwood J.,Jacob L.,Johnson D.,Jones S.,de Jong P.J.,Joseph S.S.,Keenan S.,Kelly S.,Kershaw J.K.,Khan Z.,Kioschis P.,Klages S.,Knights A.J.,Kosiura A.,Kovar-Smith C.,Laird G.K.,Langford C.,Lawlor S.,Leversha M.,Lewis L.,Liu W.,Lloyd C.,Lloyd D.M.,Loulseged H.,Loveland J.E.,Lovell J.D.,Lozado R.,Lu J.,Lyne R.,Ma J.,Maheshwari M.,Matthews L.H.,McDowall J.,McLaren S.,McMurray A.,Meidl P.,Meitinger T.,Milne S.,Miner G.,Mistry S.L.,Morgan M.,Morris S.,Mueller I.,Mullikin J.C.,Nguyen N.,Nordsiek G.,Nyakatura G.,O'dell C.N.,Okwuonu G.,Palmer S.,Pandian R.,Parker D.,Parrish J.,Pasternak S.,Patel D.,Pearce A.V.,Pearson D.M.,Pelan S.E.,Perez L.,Porter K.M.,Ramsey Y.,Reichwald K.,Rhodes S.,Ridler K.A.,Schlessinger D.,Schueler M.G.,Sehra H.K.,Shaw-Smith C.,Shen H.,Sheridan E.M.,Shownkeen R.,Skuce C.D.,Smith M.L.,Sotheran E.C.,Steingruber H.E.,Steward C.A.,Storey R.,Swann R.M.,Swarbreck D.,Tabor P.E.,Taudien S.,Taylor T.,Teague B.,Thomas K.,Thorpe A.,Timms K.,Tracey A.,Trevanion S.,Tromans A.C.,d'Urso M.,Verduzco D.,Villasana D.,Waldron L.,Wall M.,Wang Q.,Warren J.,Warry G.L.,Wei X.,West A.,Whitehead S.L.,Whiteley M.N.,Wilkinson J.E.,Willey D.L.,Williams G.,Williams L.,Williamson A.,Williamson H.,Wilming L.,Woodmansey R.L.,Wray P.W.,Yen J.,Zhang J.,Zhou J.,Zoghbi H.,Zorilla S.,Buck D.,Reinhardt R.,Poustka A.,Rosenthal A.,Lehrach H.,Meindl A.,Minx P.J.,Hillier L.W.,Willard H.F.,Wilson R.K.,Waterston R.H.,Rice C.M.,Vaudin M.,Coulson A.,Nelson D.L.,Weinstock G.,Sulston J.E.,Durbin R.M.,Hubbard T.,Gibbs R.A.,Beck S.,Rogers J.,Bentley D.R.
The DNA sequence of the human X chromosome.
Nature
434
325-337
2005
580265
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
580266
Candat A.,Poupart P.,Andrieu J.P.,Chevrollier A.,Reynier P.,Rogniaux H.,Avelange-Macherel M.H.,Macherel D.
Experimental determination of organelle targeting-peptide cleavage sites using transient expression of green fluorescent protein translational fusions.
Anal. Biochem.
434
44-51
2013
580267
Harris E.E.,Hey J.
X chromosome evidence for ancient human histories.
Proc. Natl. Acad. Sci. U.S.A.
96
3320-3324
1999
580268
Ito M.,Huq A.H.,Naito E.,Saijo T.,Takeda E.,Kuroda Y.
Mutation of E1 alpha gene in a female patient with pyruvate dehydrogenase deficiency due to rapid degradation of E1 protein.
J. Inherit. Metab. Dis.
15
848-856
1992
580269
Korotchkina L.G.,Patel M.S.
Mutagenesis studies of the phosphorylation sites of recombinant human pyruvate dehydrogenase. Site-specific regulation.
J. Biol. Chem.
270
14297-14304
1995
580270
Korotchkina L.G.,Patel M.S.
Site specificity of four pyruvate dehydrogenase kinase isoenzymes toward the three phosphorylation sites of human pyruvate dehydrogenase.
J. Biol. Chem.
276
37223-37229
2001
580271
Hiromasa Y.,Fujisawa T.,Aso Y.,Roche T.E.
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
580272
Olsen J.V.,Blagoev B.,Gnad F.,Macek B.,Kumar C.,Mortensen P.,Mann M.
Global, in vivo, and site-specific phosphorylation dynamics in signaling networks.
Cell
127
635-648
2006
580273
Zahedi R.P.,Lewandrowski U.,Wiesner J.,Wortelkamp S.,Moebius J.,Schuetz C.,Walter U.,Gambaryan S.,Sickmann A.
Phosphoproteome of resting human platelets.
J. Proteome Res.
7
526-534
2008
580274
Daub H.,Olsen J.V.,Bairlein M.,Gnad F.,Oppermann F.S.,Korner R.,Greff Z.,Keri G.,Stemmann O.,Mann M.
Kinase-selective enrichment enables quantitative phosphoproteomics of the kinome across the cell cycle.
Mol. Cell
31
438-448
2008
580275
Dephoure N.,Zhou C.,Villen J.,Beausoleil S.A.,Bakalarski C.E.,Elledge S.J.,Gygi S.P.
A quantitative atlas of mitotic phosphorylation.
Proc. Natl. Acad. Sci. U.S.A.
105
10762-10767
2008
580276
Gauci S.,Helbig A.O.,Slijper M.,Krijgsveld J.,Heck A.J.,Mohammed S.
Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach.
Anal. Chem.
81
4493-4501
2009
580277
Choudhary C.,Kumar C.,Gnad F.,Nielsen M.L.,Rehman M.,Walther T.C.,Olsen J.V.,Mann M.
Lysine acetylation targets protein complexes and co-regulates major cellular functions.
Science
325
834-840
2009
580278
Olsen J.V.,Vermeulen M.,Santamaria A.,Kumar C.,Miller M.L.,Jensen L.J.,Gnad F.,Cox J.,Jensen T.S.,Nigg E.A.,Brunak S.,Mann M.
Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis.
Sci. Signal.
3
0-0
2010
580279
Burkard T.R.,Planyavsky M.,Kaupe I.,Breitwieser F.P.,Buerckstuemmer T.,Bennett K.L.,Superti-Furga G.,Colinge J.
Initial characterization of the human central proteome.
BMC Syst. Biol.
5
17-17
2011
580280
Rigbolt K.T.,Prokhorova T.A.,Akimov V.,Henningsen J.,Johansen P.T.,Kratchmarova I.,Kassem M.,Mann M.,Olsen J.V.,Blagoev B.
System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation.
Sci. Signal.
4
0-0
2011
580281
Zhou H.,Di Palma S.,Preisinger C.,Peng M.,Polat A.N.,Heck A.J.,Mohammed S.
Toward a comprehensive characterization of a human cancer cell phosphoproteome.
J. Proteome Res.
12
260-271
2013
580282
Bian Y.,Song C.,Cheng K.,Dong M.,Wang F.,Huang J.,Sun D.,Wang L.,Ye M.,Zou H.
An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
J. Proteomics
96
253-262
2014
580283
Vaca Jacome A.S.,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
15
2519-2524
2015
580284
Ciszak E.M.,Korotchkina L.G.,Dominiak P.M.,Sidhu S.,Patel M.S.
Structural basis for flip-flop action of thiamin pyrophosphate-dependent enzymes revealed by human pyruvate dehydrogenase.
J. Biol. Chem.
278
21240-21246
2003
580285
Dahl H.-H.M.,Brown G.K.,Brown R.M.,Hansen L.L.,Kerr D.S.,Wexler I.D.,Patel M.S.,de Meirleir L.,Lissens W.,Chun K.,McKay N.,Robinson B.H.
Mutations and polymorphisms in the pyruvate dehydrogenase E1 alpha gene.
Hum. Mutat.
1
97-102
1992
580286
Seifert F.,Ciszak E.,Korotchkina L.,Golbik R.,Spinka M.,Dominiak P.,Sidhu S.,Brauer J.,Patel M.S.,Tittmann K.
Phosphorylation of serine 264 impedes active site accessibility in the E1 component of the human pyruvate dehydrogenase multienzyme complex.
Biochemistry
46
6277-6287
2007
580287
Kato M.,Wynn R.M.,Chuang J.L.,Tso S.C.,Machius M.,Li J.,Chuang D.T.
Structural basis for inactivation of the human pyruvate dehydrogenase complex by phosphorylation: role of disordered phosphorylation loops.
Structure
16
1849-1859
2008
580288
Hansen L.L.,Brown G.K.,Kirby D.M.,Dahl H.-H.M.
Characterization of the mutations in three patients with pyruvate dehydrogenase E1 alpha deficiency.
J. Inherit. Metab. Dis.
14
140-151
1991
580289
De Meirleir L.,Lissens W.,Vamos E.,Liebaers I.
Pyruvate dehydrogenase (PDH) deficiency caused by a 21-base pair insertion mutation in the E1 alpha subunit.
Hum. Genet.
88
649-652
1992
580290
Dahl H.-H.M.,Hansen L.L.,Brown R.M.,Danks D.M.,Rogers J.G.,Brown G.K.
X-linked pyruvate dehydrogenase E1 alpha subunit deficiency in heterozygous females: variable manifestation of the same mutation.
J. Inherit. Metab. Dis.
15
835-847
1992
580291
Matthews P.M.,Marchington D.R.,Squier M.,Land J.,Brown R.M.,Brown G.K.
Molecular genetic characterization of an X-linked form of Leigh's syndrome.
Ann. Neurol.
33
652-655
1993
580292
Chun K.,McKay N.,Petrova-Benedict R.,Robinson B.H.
Mutations in the X-linked E1 alpha subunit of pyruvate dehydrogenase leading to deficiency of the pyruvate dehydrogenase complex.
Hum. Mol. Genet.
2
449-454
1993
580293
Matthews P.M.,Brown R.M.,Otero L.J.,Marchington D.R.,LeGris M.,Howes R.,Meadows L.S.,Shevell M.,Scriver C.R.,Brown G.K.
Pyruvate dehydrogenase deficiency. Clinical presentation and molecular genetic characterization of five new patients.
Brain
117
435-443
1994
580294
Hansen L.L.,Horn N.,Dahl H.-H.M.,Kruse T.A.
Pyruvate dehydrogenase deficiency caused by a 33 base pair duplication in the PDH E1 alpha subunit.
Hum. Mol. Genet.
3
1021-1022
1994
580295
Dahl H.-H.M.,Brown G.K.
Pyruvate dehydrogenase deficiency in a male caused by a point mutation (F205L) in the E1 alpha subunit.
Hum. Mutat.
3
152-155
1994
580296
Awata H.,Endo F.,Tanoue A.,Kitano A.,Matsuda I.
Characterization of a point mutation in the pyruvate dehydrogenase E1 alpha gene from two boys with primary lactic acidaemia.
J. Inherit. Metab. Dis.
17
189-195
1994
580297
Chun K.,MacKay N.,Petrova-Benedict R.,Federico A.,Fois A.,Cole D.E.C.,Robertson E.,Robinson B.H.
Mutations in the X-linked E1 alpha subunit of pyruvate dehydrogenase: exon skipping, insertion of duplicate sequence, and missense mutations leading to the deficiency of the pyruvate dehydrogenase complex.
Am. J. Hum. Genet.
56
558-569
1995
580298
Takakubo F.,Cartwright P.,Hoogenraad N.,Thorburn D.R.,Collins F.,Lithgow T.,Dahl H.-H.M.
An amino acid substitution in the pyruvate dehydrogenase E1 alpha gene, affecting mitochondrial import of the precursor protein.
Am. J. Hum. Genet.
57
772-780
1995
580299
Hemalatha S.G.,Kerr D.S.,Wexler I.D.,Lusk M.M.,Kaung M.,Du Y.,Kolli M.,Schelper R.L.,Patel M.S.
Pyruvate dehydrogenase complex deficiency due to a point mutation (P188L) within the thiamine pyrophosphate binding loop of the E1 alpha subunit.
Hum. Mol. Genet.
4
315-318
1995
580300
Lissens W.,de Meirleir L.,Seneca S.,Benelli C.,Marsac C.,Poll-The B.T.,Briones P.,Ruitenbeek W.,van Diggelen O.,Chaigne D.,Ramaekers V.,Liebaers I.
Mutation analysis of the pyruvate dehydrogenase E1 alpha gene in eight patients with a pyruvate dehydrogenase complex deficiency.
Hum. Mutat.
7
46-51
1996
580301
Tripatara A.,Kerr D.S.,Lusk M.M.,Kolli M.,Tan J.,Patel M.S.
Three new mutations of the pyruvate dehydrogenase alpha subunit: a point mutation (M181V), 3 bp deletion (-R282), and 16 bp insertion/frameshift (K358SVS-->TVDQS).
Hum. Mutat.
8
180-182
1996
580302
Naito E.,Ito M.,Yokota I.,Saijo T.,Matsuda J.,Osaka H.,Kimura S.,Kuroda Y.
Biochemical and molecular analysis of an X-linked case of Leigh syndrome associated with thiamin-responsive pyruvate dehydrogenase deficiency.
J. Inherit. Metab. Dis.
20
539-548
1997
580303
Otero L.J.,Brown R.M.,Brown G.K.
Arginine 302 mutations in the pyruvate dehydrogenase E1alpha subunit gene: identification of further patients and in vitro demonstration of pathogenicity.
Hum. Mutat.
12
114-121
1998
580304
de Ligt J.,Willemsen M.H.,van Bon B.W.,Kleefstra T.,Yntema H.G.,Kroes T.,Vulto-van Silfhout A.T.,Koolen D.A.,de Vries P.,Gilissen C.,del Rosario M.,Hoischen A.,Scheffer H.,de Vries B.B.,Brunner H.G.,Veltman J.A.,Vissers L.E.
Diagnostic exome sequencing in persons with severe intellectual disability.
N. Engl. J. Med.
367
1921-1929
2012
580305
Parrini E.,Marini C.,Mei D.,Galuppi A.,Cellini E.,Pucatti D.,Chiti L.,Rutigliano D.,Bianchini C.,Virdo S.,De Vita D.,Bigoni S.,Barba C.,Mari F.,Montomoli M.,Pisano T.,Rosati A.,Guerrini R.
Diagnostic targeted resequencing in 349 patients with drug-resistant pediatric epilepsies identifies causative mutations in 30 different genes.
Hum. Mutat.
38
216-225
2017
