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Sequence of ACADV_HUMAN

EC Number:1.3.8.9

EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
very-long-chain acyl-CoA dehydrogenase
P49748
Homo sapiens
655
70390
Reaction
a very-long-chain acyl-CoA + electron-transfer flavoprotein = a very-long-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein
Other sequences found for EC No. 1.3.8.9

General information:

Sequence
show sequence in fasta format
  0 MQAARMAASL GRQLLRLGGG SSRLTALLGQ PRPGPARRPY AGGAAQLALD KSDSHPSDAL
 60 TRKKPAKAES KSFAVGMFKG QLTTDQVFPY PSVLNEEQTQ FLKELVEPVS RFFEEVNDPA
120 KNDALEMVEE TTWQGLKELG AFGLQVPSEL GGVGLCNTQY ARLVEIVGMH DLGVGITLGA
180 HQSIGFKGIL LFGTKAQKEK YLPKLASGET VAAFCLTEPS SGSDAASIRT SAVPSPCGKY
240 YTLNGSKLWI SNGGLADIFT VFAKTPVTDP ATGAVKEKIT AFVVERGFGG ITHGPPEKKM
300 GIKASNTAEV FFDGVRVPSE NVLGEVGSGF KVAMHILNNG RFGMAAALAG TMRGIIAKAV
360 DHATNRTQFG EKIHNFGLIQ EKLARMVMLQ YVTESMAYMV SANMDQGATD FQIEAAISKI
420 FGSEAAWKVT DECIQIMGGM GFMKEPGVER VLRDLRIFRI FEGTNDILRL FVALQGCMDK
480 GKELSGLGSA LKNPFGNAGL LLGEAGKQLR RRAGLGSGLS LSGLVHPELS RSGELAVRAL
540 EQFATVVEAK LIKHKKGIVN EQFLLQRLAD GAIDLYAMVV VLSRASRSLS EGHPTAQHEK
600 MLCDTWCIEA AARIREGMAA LQSDPWQQEL YRNFKSISKA LVERGGVVTS NPLGF
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Sequence related references
Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
894102
Aoyama T.,Souri M.,Ueno I.,Kamijo T.,Yamaguchi S.,Rhead W.J.,Tanaka K.,Hashimoto T.
Cloning of human very-long-chain acyl-coenzyme A dehydrogenase and molecular characterization of its deficiency in two patients.
Am. J. Hum. Genet.
57
273-283
1995
894103
Andresen B.S.,Bross P.,Vianey-Saban C.,Divry P.,Zabot M.-T.,Roe C.R.,Nada M.A.,Byskov A.,Kruse T.A.,Neve S.,Kristiansen K.,Knudsen I.,Corydon M.J.,Gregersen N.
Cloning and characterization of human very-long-chain acyl-CoA dehydrogenase cDNA, chromosomal assignment of the gene and identification in four patients of nine different mutations within the VLCAD gene.
Hum. Mol. Genet.
5
461-472
1996
894104
Orii K.O.,Aoyama T.,Souri M.,Orii K.E.,Kondo N.,Orii T.,Hashimoto T.
Genomic DNA organization of human mitochondrial very-long-chain acyl-CoA dehydrogenase and mutation analysis.
Biochem. Biophys. Res. Commun.
217
987-992
1995
894105
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
894106
Zody M.C.,Garber M.,Adams D.J.,Sharpe T.,Harrow J.,Lupski J.R.,Nicholson C.,Searle S.M.,Wilming L.,Young S.K.,Abouelleil A.,Allen N.R.,Bi W.,Bloom T.,Borowsky M.L.,Bugalter B.E.,Butler J.,Chang J.L.,Chen C.-K.,Cook A.,Corum B.,Cuomo C.A.,de Jong P.J.,DeCaprio D.,Dewar K.,FitzGerald M.,Gilbert J.,Gibson R.,Gnerre S.,Goldstein S.,Grafham D.V.,Grocock R.,Hafez N.,Hagopian D.S.,Hart E.,Norman C.H.,Humphray S.,Jaffe D.B.,Jones M.,Kamal M.,Khodiyar V.K.,LaButti K.,Laird G.,Lehoczky J.,Liu X.,Lokyitsang T.,Loveland J.,Lui A.,Macdonald P.,Major J.E.,Matthews L.,Mauceli E.,McCarroll S.A.,Mihalev A.H.,Mudge J.,Nguyen C.,Nicol R.,O'Leary S.B.,Osoegawa K.,Schwartz D.C.,Shaw-Smith C.,Stankiewicz P.,Steward C.,Swarbreck D.,Venkataraman V.,Whittaker C.A.,Yang X.,Zimmer A.R.,Bradley A.,Hubbard T.,Birren B.W.,Rogers J.,Lander E.S.,Nusbaum C.
DNA sequence of human chromosome 17 and analysis of rearrangement in the human lineage.
Nature
440
1045-1049
2006
894107
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
894108
Aoyama T.,Souri M.,Ushikubo S.,Kamijo T.,Yamaguchi S.,Kelley R.I.,Rhead W.J.,Uetake K.,Tanaka K.,Hashimoto T.
Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients.
J. Clin. Invest.
95
2465-2473
1995
894109
Souri M.,Aoyama T.,Cox G.F.,Hashimoto T.
Catalytic and FAD-binding residues of mitochondrial very long chain acyl-coenzyme A dehydrogenase.
J. Biol. Chem.
273
4227-4231
1998
894110
Andresen B.S.,Olpin S.,Poorthuis B.J.H.M.,Scholte H.R.,Vianey-Saban C.,Wanders R.,Ijlst L.,Morris A.,Pourfarzam M.,Bartlett K.,Baumgartner E.R.,de Klerk J.B.C.,Schroeder L.D.,Corydon T.J.,Lund H.,Winter V.,Bross P.,Bolund L.,Gregersen N.
Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency.
Am. J. Hum. Genet.
64
479-494
1999
894111
Goetzman E.S.,Wang Y.,He M.,Mohsen A.W.,Ninness B.K.,Vockley J.
Expression and characterization of mutations in human very long-chain acyl-CoA dehydrogenase using a prokaryotic system.
Mol. Genet. Metab.
91
138-147
2007
894112
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
894113
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
894114
He M.,Pei Z.,Mohsen A.W.,Watkins P.,Murdoch G.,Van Veldhoven P.P.,Ensenauer R.,Vockley J.
Identification and characterization of new long chain acyl-CoA dehydrogenases.
Mol. Genet. Metab.
102
418-429
2011
894115
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
894116
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
894117
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
894119
McAndrew R.P.,Wang Y.,Mohsen A.W.,He M.,Vockley J.,Kim J.J.
Structural basis for substrate fatty acyl chain specificity: crystal structure of human very-long-chain acyl-CoA dehydrogenase.
J. Biol. Chem.
283
9435-9443
2008
894120
Souri M.,Aoyama T.,Orii K.,Yamaguchi S.,Hashimoto T.
Mutation analysis of very-long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency: identification and characterization of mutant VLCAD cDNAs from four patients.
Am. J. Hum. Genet.
58
97-106
1996
894121
Smelt A.H.,Poorthuis B.J.H.M.,Onkenhout W.,Scholte H.R.,Andresen B.S.,van Duinen S.G.,Gregersen N.,Wintzen A.R.
Very long chain acyl-coenzyme A dehydrogenase deficiency with adult onset.
Ann. Neurol.
43
540-544
1998
894122
Souri M.,Aoyama T.,Yamaguchi S.,Hashimoto T.
Relationship between structure and substrate-chain-length specificity of mitochondrial very-long-chain acyl-coenzyme A dehydrogenase.
Eur. J. Biochem.
257
592-598
1998
894123
Souri M.,Aoyama T.,Hoganson G.,Hashimoto T.
Very-long-chain acyl-CoA dehydrogenase subunit assembles to the dimer form on mitochondrial inner membrane.
FEBS Lett.
426
187-190
1998
894124
Mathur A.,Sims H.F.,Gopalakrishnan D.,Gibson B.,Rinaldo P.,Vockley J.,Hug G.,Strauss A.W.
Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death.
Circulation
99
1337-1343
1999