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Sequence of DLDH_HUMAN

EC Number:1.2.1.104

EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
pyruvate dehydrogenase system
P09622
Homo sapiens
509
54177
Reaction
pyruvate + CoA + NAD+ = acetyl-CoA + CO2 + NADH
Other sequences found for EC No. 1.2.1.104

EC Number:1.8.1.4

EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
dihydrolipoyl dehydrogenase
P09622
Homo sapiens
509
54177
Reaction
protein N6-(dihydrolipoyl)lysine + NAD+ = protein N6-(lipoyl)lysine + NADH + H+
Other sequences found for EC No. 1.8.1.4

EC Number:2.3.1.168

EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
dihydrolipoyllysine-residue (2-methylpropanoyl)transferase
P09622
Homo sapiens
509
54177
Reaction
2-methylpropanoyl-CoA + enzyme N6-(dihydrolipoyl)lysine = CoA + enzyme N6-(S-[2-methylpropanoyl]dihydrolipoyl)lysine
Other sequences found for EC No. 2.3.1.168

General information:

Sequence
show sequence in fasta format
  0 MQSWSRVYCS LAKRGHFNRI SHGLQGLSAV PLRTYADQPI DADVTVIGSG PGGYVAAIKA
 60 AQLGFKTVCI EKNETLGGTC LNVGCIPSKA LLNNSHYYHM AHGKDFASRG IEMSEVRLNL
120 DKMMEQKSTA VKALTGGIAH LFKQNKVVHV NGYGKITGKN QVTATKADGG TQVIDTKNIL
180 IATGSEVTPF PGITIDEDTI VSSTGALSLK KVPEKMVVIG AGVIGVELGS VWQRLGADVT
240 AVEFLGHVGG VGIDMEISKN FQRILQKQGF KFKLNTKVTG ATKKSDGKID VSIEAASGGK
300 AEVITCDVLL VCIGRRPFTK NLGLEELGIE LDPRGRIPVN TRFQTKIPNI YAIGDVVAGP
360 MLAHKAEDEG IICVEGMAGG AVHIDYNCVP SVIYTHPEVA WVGKSEEQLK EEGIEYKVGK
420 FPFAANSRAK TNADTDGMVK ILGQKSTDRV LGAHILGPGA GEMVNEAALA LEYGASCEDI
480 ARVCHAHPTL SEAFREANLA ASFGKSINF
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Sequence related references
Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
410109
Otulakowski G.,Robinson B.H.
Isolation and sequence determination of cDNA clones for porcine and human lipoamide dehydrogenase. Homology to other disulfide oxidoreductases.
J. Biol. Chem.
262
17313-17318
1987
410110
Pons G.,Raefsky-Estrin C.,Carothers D.J.,Pepin R.A.,Javed A.A.,Jesse B.W.,Ganapathi M.K.,Samols D.,Patel M.S.
Cloning and cDNA sequence of the dihydrolipoamide dehydrogenase component human alpha-ketoacid dehydrogenase complexes.
Proc. Natl. Acad. Sci. U.S.A.
85
1422-1426
1988
410111
Feigenbaum A.S.,Robinson B.H.
The structure of the human dihydrolipoamide dehydrogenase gene (DLD) and its upstream elements.
Genomics
17
376-381
1993
410112
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
410114
Hillier L.W.,Fulton R.S.,Fulton L.A.,Graves T.A.,Pepin K.H.,Wagner-McPherson C.,Layman D.,Maas J.,Jaeger S.,Walker R.,Wylie K.,Sekhon M.,Becker M.C.,O'Laughlin M.D.,Schaller M.E.,Fewell G.A.,Delehaunty K.D.,Miner T.L.,Nash W.E.,Cordes M.,Du H.,Sun H.,Edwards J.,Bradshaw-Cordum H.,Ali J.,Andrews S.,Isak A.,Vanbrunt A.,Nguyen C.,Du F.,Lamar B.,Courtney L.,Kalicki J.,Ozersky P.,Bielicki L.,Scott K.,Holmes A.,Harkins R.,Harris A.,Strong C.M.,Hou S.,Tomlinson C.,Dauphin-Kohlberg S.,Kozlowicz-Reilly A.,Leonard S.,Rohlfing T.,Rock S.M.,Tin-Wollam A.-M.,Abbott A.,Minx P.,Maupin R.,Strowmatt C.,Latreille P.,Miller N.,Johnson D.,Murray J.,Woessner J.P.,Wendl M.C.,Yang S.-P.,Schultz B.R.,Wallis J.W.,Spieth J.,Bieri T.A.,Nelson J.O.,Berkowicz N.,Wohldmann P.E.,Cook L.L.,Hickenbotham M.T.,Eldred J.,Williams D.,Bedell J.A.,Mardis E.R.,Clifton S.W.,Chissoe S.L.,Marra M.A.,Raymond C.,Haugen E.,Gillett W.,Zhou Y.,James R.,Phelps K.,Iadanoto S.,Bubb K.,Simms E.,Levy R.,Clendenning J.,Kaul R.,Kent W.J.,Furey T.S.,Baertsch R.A.,Brent M.R.,Keibler E.,Flicek P.,Bork P.,Suyama M.,Bailey J.A.,Portnoy M.E.,Torrents D.,Chinwalla A.T.,Gish W.R.,Eddy S.R.,McPherson J.D.,Olson M.V.,Eichler E.E.,Green E.D.,Waterston R.H.,Wilson R.K.
The DNA sequence of human chromosome 7.
Nature
424
157-164
2003
410115
Scherer S.W.,Cheung J.,MacDonald J.R.,Osborne L.R.,Nakabayashi K.,Herbrick J.-A.,Carson A.R.,Parker-Katiraee L.,Skaug J.,Khaja R.,Zhang J.,Hudek A.K.,Li M.,Haddad M.,Duggan G.E.,Fernandez B.A.,Kanematsu E.,Gentles S.,Christopoulos C.C.,Choufani S.,Kwasnicka D.,Zheng X.H.,Lai Z.,Nusskern D.R.,Zhang Q.,Gu Z.,Lu F.,Zeesman S.,Nowaczyk M.J.,Teshima I.,Chitayat D.,Shuman C.,Weksberg R.,Zackai E.H.,Grebe T.A.,Cox S.R.,Kirkpatrick S.J.,Rahman N.,Friedman J.M.,Heng H.H.Q.,Pelicci P.G.,Lo-Coco F.,Belloni E.,Shaffer L.G.,Pober B.,Morton C.C.,Gusella J.F.,Bruns G.A.P.,Korf B.R.,Quade B.J.,Ligon A.H.,Ferguson H.,Higgins A.W.,Leach N.T.,Herrick S.R.,Lemyre E.,Farra C.G.,Kim H.-G.,Summers A.M.,Gripp K.W.,Roberts W.,Szatmari P.,Winsor E.J.T.,Grzeschik K.-H.,Teebi A.,Minassian B.A.,Kere J.,Armengol L.,Pujana M.A.,Estivill X.,Wilson M.D.,Koop B.F.,Tosi S.,Moore G.E.,Boright A.P.,Zlotorynski E.,Kerem B.,Kroisel P.M.,Petek E.,Oscier D.G.,Mould S.J.,Doehner H.,Doehner K.,Rommens J.M.,Vincent J.B.,Venter J.C.,Li P.W.,Mural R.J.,Adams M.D.,Tsui L.-C.
Human chromosome 7: DNA sequence and biology.
Science
300
767-772
2003
410117
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
410118
Johanning G.L.,Morris J.I.,Madhusudhan K.T.,Samols D.,Patel M.S.
Characterization of the transcriptional regulatory region of the human dihydrolipoamide dehydrogenase gene.
Proc. Natl. Acad. Sci. U.S.A.
89
10964-10968
1992
410119
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
410120
Mitra K.,Rangaraj N.,Shivaji S.
Novelty of the pyruvate metabolic enzyme dihydrolipoamide dehydrogenase in spermatozoa: correlation of its localization, tyrosine phosphorylation, and activity during sperm capacitation.
J. Biol. Chem.
280
25743-25753
2005
410121
Babady N.E.,Pang Y.P.,Elpeleg O.,Isaya G.
Cryptic proteolytic activity of dihydrolipoamide dehydrogenase.
Proc. Natl. Acad. Sci. U.S.A.
104
6158-6163
2007
410122
Patel M.S.,Korotchkina L.G.,Sidhu S.
Interaction of E1 and E3 components with the core proteins of the human pyruvate dehydrogenase complex.
J. Mol. Catal., B Enzym.
61
2-6
2009
410123
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
410124
Park Y.H.,Patel M.S.
Characterization of interactions of dihydrolipoamide dehydrogenase with its binding protein in the human pyruvate dehydrogenase complex.
Biochem. Biophys. Res. Commun.
395
416-419
2010
410125
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
410126
Rosenow A.,Noben J.P.,Jocken J.,Kallendrusch S.,Fischer-Posovszky P.,Mariman E.C.,Renes J.
Resveratrol-induced changes of the human adipocyte secretion profile.
J. Proteome Res.
11
4733-4743
2012
410127
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
410128
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
410129
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
410130
Wang Y.,Guo Y.R.,Liu K.,Yin Z.,Liu R.,Xia Y.,Tan L.,Yang P.,Lee J.H.,Li X.J.,Hawke D.,Zheng Y.,Qian X.,Lyu J.,He J.,Xing D.,Tao Y.J.,Lu Z.
KAT2A coupled with the alpha-KGDH complex acts as a histone H3 succinyltransferase.
Nature
552
273-277
2017
410131
Brautigam C.A.,Chuang J.L.,Tomchick D.R.,Machius M.,Chuang D.T.
Crystal structure of human dihydrolipoamide dehydrogenase: NAD+/NADH binding and the structural basis of disease-causing mutations.
J. Mol. Biol.
350
543-552
2005
410132
Ciszak E.M.,Makal A.,Hong Y.S.,Vettaikkorumakankauv A.K.,Korotchkina L.G.,Patel M.S.
How dihydrolipoamide dehydrogenase-binding protein binds dihydrolipoamide dehydrogenase in the human pyruvate dehydrogenase complex.
J. Biol. Chem.
281
648-655
2006
410133
Brautigam C.A.,Wynn R.M.,Chuang J.L.,Machius M.,Tomchick D.R.,Chuang D.T.
Structural insight into interactions between dihydrolipoamide dehydrogenase (E3) and E3 binding protein of human pyruvate dehydrogenase complex.
Structure
14
611-621
2006
410134
Vijayakrishnan S.,Kelly S.M.,Gilbert R.J.,Callow P.,Bhella D.,Forsyth T.,Lindsay J.G.,Byron O.
Solution structure and characterisation of the human pyruvate dehydrogenase complex core assembly.
J. Mol. Biol.
399
71-93
2010
410135
Liu T.-C.,Kim H.,Arizmendi C.,Kitano A.,Patel M.S.
Identification of two missense mutations in a dihydrolipoamide dehydrogenase-deficient patient.
Proc. Natl. Acad. Sci. U.S.A.
90
5186-5190
1993
410136
Hong Y.S.,Kerr D.S.,Liu T.C.,Lusk M.,Powell B.R.,Patel M.S.
Deficiency of dihydrolipoamide dehydrogenase due to two mutant alleles (E340K and G101del). Analysis of a family and prenatal testing.
Biochim. Biophys. Acta
1362
160-168
1997
410137
Hong Y.S.,Kerr D.S.,Craigen W.J.,Tan J.,Pan Y.,Lusk M.,Patel M.S.
Identification of two mutations in a compound heterozygous child with dihydrolipoamide dehydrogenase deficiency.
Hum. Mol. Genet.
5
1925-1930
1996
410138
Shany E.,Saada A.,Landau D.,Shaag A.,Hershkovitz E.,Elpeleg O.N.
Lipoamide dehydrogenase deficiency due to a novel mutation in the interface domain.
Biochem. Biophys. Res. Commun.
262
163-166
1999
410139
Cerna L.,Wenchich L.,Hansikova H.,Kmoch S.,Peskova K.,Chrastina P.,Brynda J.,Zeman J.
Novel mutations in a boy with dihydrolipoamide dehydrogenase deficiency.
Med. Sci. Monit.
7
1319-1325
2001
410140
Grafakou O.,Oexle K.,van den Heuvel L.,Smeets R.,Trijbels F.,Goebel H.H.,Bosshard N.,Superti-Furga A.,Steinmann B.,Smeitink J.
Leigh syndrome due to compound heterozygosity of dihydrolipoamide dehydrogenase gene mutations. Description of the first E3 splice site mutation.
Eur. J. Pediatr.
162
714-718
2003
410141
Odievre M.H.,Chretien D.,Munnich A.,Robinson B.H.,Dumoulin R.,Masmoudi S.,Kadhom N.,Roetig A.,Rustin P.,Bonnefont J.P.
A novel mutation in the dihydrolipoamide dehydrogenase E3 subunit gene (DLD) resulting in an atypical form of alpha-ketoglutarate dehydrogenase deficiency.
Hum. Mutat.
25
323-324
2005
410142
Cameron J.M.,Levandovskiy V.,Mackay N.,Raiman J.,Renaud D.L.,Clarke J.T.,Feigenbaum A.,Elpeleg O.,Robinson B.H.
Novel mutations in dihydrolipoamide dehydrogenase deficiency in two cousins with borderline-normal PDH complex activity.
Am. J. Med. Genet. A
140
1542-1552
2006
410143
Shaag A.,Saada A.,Berger I.,Mandel H.,Joseph A.,Feigenbaum A.,Elpeleg O.N.
Molecular basis of lipoamide dehydrogenase deficiency in Ashkenazi Jews.
Am. J. Med. Genet.
82
177-182
1999