Sequence of ODBB_HUMAN
EC Number:1.2.4.4
EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)
P21953
Homo sapiens
392
43122
Reaction
3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 + 2 H+
Other sequences found for EC No. 1.2.4.4
General information:
Sequence
0 MAVVAAAAGW LLRLRAAGAE GHWRRLPGAG LARGFLHPAA TVEDAAQRRQ VAHFTFQPDP
60 EPREYGQTQK MNLFQSVTSA LDNSLAKDPT AVIFGEDVAF GGVFRCTVGL RDKYGKDRVF
120 NTPLCEQGIV GFGIGIAVTG ATAIAEIQFA DYIFPAFDQI VNEAAKYRYR SGDLFNCGSL
180 TIRSPWGCVG HGALYHSQSP EAFFAHCPGI KVVIPRSPFQ AKGLLLSCIE DKNPCIFFEP
240 KILYRAAAEE VPIEPYNIPL SQAEVIQEGS DVTLVAWGTQ VHVIREVASM AKEKLGVSCE
300 VIDLRTIIPW DVDTICKSVI KTGRLLISHE APLTGGFASE ISSTVQEECF LNLEAPISRV
360 CGYDTPFPHI FEPFYIPDKW KCYDALRKMI NY
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Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
69473
Matsuda I.,Asaka J.,Akaboshi I.,Endo F.,Mitsubuchi H.,Nobukuni Y.
Maple syrup urine disease. Complete primary structure of the E1 beta subunit of human branched chain alpha-ketoacid dehydrogenase complex deduced from the nucleotide sequence and a gene analysis of patients with this disease.
J. Clin. Invest.
86
242-247
1990
69474
Chuang J.L.,Cox R.P.,Chuang D.T.
Maple syrup urine disease: the E1beta gene of human branched-chain alpha-ketoacid dehydrogenase complex has 11 rather than 10 exons, and the 3' UTR in one of the two E1beta mRNAs arises from intronic sequences.
Am. J. Hum. Genet.
58
1373-1377
1996
69475
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
69477
Mungall A.J.,Palmer S.A.,Sims S.K.,Edwards C.A.,Ashurst J.L.,Wilming L.,Jones M.C.,Horton R.,Hunt S.E.,Scott C.E.,Gilbert J.G.R.,Clamp M.E.,Bethel G.,Milne S.,Ainscough R.,Almeida J.P.,Ambrose K.D.,Andrews T.D.,Ashwell R.I.S.,Babbage A.K.,Bagguley C.L.,Bailey J.,Banerjee R.,Barker D.J.,Barlow K.F.,Bates K.,Beare D.M.,Beasley H.,Beasley O.,Bird C.P.,Blakey S.E.,Bray-Allen S.,Brook J.,Brown A.J.,Brown J.Y.,Burford D.C.,Burrill W.,Burton J.,Carder C.,Carter N.P.,Chapman J.C.,Clark S.Y.,Clark G.,Clee C.M.,Clegg S.,Cobley V.,Collier R.E.,Collins J.E.,Colman L.K.,Corby N.R.,Coville G.J.,Culley K.M.,Dhami P.,Davies J.,Dunn M.,Earthrowl M.E.,Ellington A.E.,Evans K.A.,Faulkner L.,Francis M.D.,Frankish A.,Frankland J.,French L.,Garner P.,Garnett J.,Ghori M.J.,Gilby L.M.,Gillson C.J.,Glithero R.J.,Grafham D.V.,Grant M.,Gribble S.,Griffiths C.,Griffiths M.N.D.,Hall R.,Halls K.S.,Hammond S.,Harley J.L.,Hart E.A.,Heath P.D.,Heathcott R.,Holmes S.J.,Howden P.J.,Howe K.L.,Howell G.R.,Huckle E.,Humphray S.J.,Humphries M.D.,Hunt A.R.,Johnson C.M.,Joy A.A.,Kay M.,Keenan S.J.,Kimberley A.M.,King A.,Laird G.K.,Langford C.,Lawlor S.,Leongamornlert D.A.,Leversha M.,Lloyd C.R.,Lloyd D.M.,Loveland J.E.,Lovell J.,Martin S.,Mashreghi-Mohammadi M.,Maslen G.L.,Matthews L.,McCann O.T.,McLaren S.J.,McLay K.,McMurray A.,Moore M.J.F.,Mullikin J.C.,Niblett D.,Nickerson T.,Novik K.L.,Oliver K.,Overton-Larty E.K.,Parker A.,Patel R.,Pearce A.V.,Peck A.I.,Phillimore B.J.C.T.,Phillips S.,Plumb R.W.,Porter K.M.,Ramsey Y.,Ranby S.A.,Rice C.M.,Ross M.T.,Searle S.M.,Sehra H.K.,Sheridan E.,Skuce C.D.,Smith S.,Smith M.,Spraggon L.,Squares S.L.,Steward C.A.,Sycamore N.,Tamlyn-Hall G.,Tester J.,Theaker A.J.,Thomas D.W.,Thorpe A.,Tracey A.,Tromans A.,Tubby B.,Wall M.,Wallis J.M.,West A.P.,White S.S.,Whitehead S.L.,Whittaker H.,Wild A.,Willey D.J.,Wilmer T.E.,Wood J.M.,Wray P.W.,Wyatt J.C.,Young L.,Younger R.M.,Bentley D.R.,Coulson A.,Durbin R.M.,Hubbard T.,Sulston J.E.,Dunham I.,Rogers J.,Beck S.
The DNA sequence and analysis of human chromosome 6.
Nature
425
805-811
2003
69479
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
69480
Chuang J.L.,Cox R.P.,Chuang D.T.
Molecular cloning of the mature E1b-beta subunit of human branched-chain alpha-keto acid dehydrogenase complex.
FEBS Lett.
262
305-309
1990
69481
Wynn R.M.,Kochi H.,Cox R.P.,Chuang D.T.
Differential processing of human and rat E1 alpha precursors of the branched-chain alpha-keto acid dehydrogenase complex caused by an N-terminal proline in the rat sequence.
Biochim. Biophys. Acta
1201
125-128
1994
69482
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
69483
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
69484
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
69485
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
69486
Nobukuni Y.,Mitsubuchi H.,Hayashida Y.,Ohta K.,Indo Y.,Ichiba Y.,Endo F.,Matsuda I.
Heterogeneity of mutations in maple syrup urine disease (MSUD): screening and identification of affected E1 alpha and E1 beta subunits of the branched-chain alpha-keto-acid dehydrogenase multienzyme complex.
Biochim. Biophys. Acta
1225
64-70
1993
69487
Edelmann L.,Wasserstein M.P.,Kornreich R.,Sansaricq C.,Snyderman S.E.,Diaz G.A.
Maple syrup urine disease: identification and carrier-frequency determination of a novel founder mutation in the Ashkenazi Jewish population.
Am. J. Hum. Genet.
69
863-868
2001
69488
Wang Y.P.,Qi M.L.,Li T.T.,Zhao Y.J.
Two novel mutations in the BCKDHB gene (R170H, Q346R) cause the classic form of maple syrup urine disease (MSUD).
Gene
498
112-115
2012
