Sequence of GCDH_HUMAN

EC Number:1.3.8.6

EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
glutaryl-CoA dehydrogenase (ETF)
Q92947
Homo sapiens
438
48127
Reaction
(E)-glutaconyl-CoA = crotonyl-CoA + CO2
Other sequences found for EC No. 1.3.8.6

EC Number:1.3.8.6

EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
glutaryl-CoA dehydrogenase (ETF)
Q92947
Homo sapiens
438
48127
Reaction
(E)-glutaconyl-CoA = crotonyl-CoA + CO2
Other sequences found for EC No. 1.3.8.6

General information:

Sequence
show sequence in fasta format
  0 MALRGVSVRL LSRGPGLHVL RTWVSSAAQT EKGGRTQSQL AKSSRPEFDW QDPLVLEEQL
 60 TTDEILIRDT FRTYCQERLM PRILLANRNE VFHREIISEM GELGVLGPTI KGYGCAGVSS
120 VAYGLLAREL ERVDSGYRSA MSVQSSLVMH PIYAYGSEEQ RQKYLPQLAK GELLGCFGLT
180 EPNSGSDPSS METRAHYNSS NKSYTLNGTK TWITNSPMAD LFVVWARCED GCIRGFLLEK
240 GMRGLSAPRI QGKFSLRASA TGMIIMDGVE VPEENVLPGA SSLGGPFGCL NNARYGIAWG
300 VLGASEFCLH TARQYALDRM QFGVPLARNQ LIQKKLADML TEITLGLHAC LQLGRLKDQD
360 KAAPEMVSLL KRNNCGKALD IARQARDMLG GNGISDEYHV IRHAMNLEAV NTYEGTHDIH
420 ALILGRAITG IQAFTASK
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Sequence related references
Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
1047018
Goodman S.I.,Kratz L.E.,Frerman F.E.
Pork and human cDNAs encoding glutaryl-CoA dehydrogenase.
Prog. Clin. Biol. Res.
375
169-173
1992
1047019
Goodman S.I.,Kratz L.E.,Digiulio K.A.,Biery B.J.,Goodman K.E.,Isaya G.,Frerman F.E.
Cloning of glutaryl-CoA dehydrogenase cDNA, and expression of wild type and mutant enzymes in Escherichia coli.
Hum. Mol. Genet.
4
1493-1498
1995
1047020
Schwartz M.,Christensen E.,Superti-Furga A.,Brandt N.J.
The human glutaryl-CoA dehydrogenase gene: report of intronic sequences and of 13 novel mutations causing glutaric aciduria type I.
Hum. Genet.
102
452-458
1998
1047022
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
1047023
Grimwood J.,Gordon L.A.,Olsen A.S.,Terry A.,Schmutz J.,Lamerdin J.E.,Hellsten U.,Goodstein D.,Couronne O.,Tran-Gyamfi M.,Aerts A.,Altherr M.,Ashworth L.,Bajorek E.,Black S.,Branscomb E.,Caenepeel S.,Carrano A.V.,Caoile C.,Chan Y.M.,Christensen M.,Cleland C.A.,Copeland A.,Dalin E.,Dehal P.,Denys M.,Detter J.C.,Escobar J.,Flowers D.,Fotopulos D.,Garcia C.,Georgescu A.M.,Glavina T.,Gomez M.,Gonzales E.,Groza M.,Hammon N.,Hawkins T.,Haydu L.,Ho I.,Huang W.,Israni S.,Jett J.,Kadner K.,Kimball H.,Kobayashi A.,Larionov V.,Leem S.-H.,Lopez F.,Lou Y.,Lowry S.,Malfatti S.,Martinez D.,McCready P.M.,Medina C.,Morgan J.,Nelson K.,Nolan M.,Ovcharenko I.,Pitluck S.,Pollard M.,Popkie A.P.,Predki P.,Quan G.,Ramirez L.,Rash S.,Retterer J.,Rodriguez A.,Rogers S.,Salamov A.,Salazar A.,She X.,Smith D.,Slezak T.,Solovyev V.,Thayer N.,Tice H.,Tsai M.,Ustaszewska A.,Vo N.,Wagner M.,Wheeler J.,Wu K.,Xie G.,Yang J.,Dubchak I.,Furey T.S.,DeJong P.,Dickson M.,Gordon D.,Eichler E.E.,Pennacchio L.A.,Richardson P.,Stubbs L.,Rokhsar D.S.,Myers R.M.,Rubin E.M.,Lucas S.M.
The DNA sequence and biology of human chromosome 19.
Nature
428
529-535
2004
1047025
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
1047026
Hyman D.B.,Tanaka K.
Specific glutaryl-CoA dehydrogenating activity is deficient in cultured fibroblasts from glutaric aciduria patients.
J. Clin. Invest.
73
778-784
1984
1047027
Goodman S.I.,Stein D.E.,Schlesinger S.,Christensen E.,Schwartz M.,Greenberg C.R.,Elpeleg O.N.
Glutaryl-CoA dehydrogenase mutations in glutaric acidemia (type I): review and report of thirty novel mutations.
Hum. Mutat.
12
141-144
1998
1047028
Rao K.S.,Albro M.,Dwyer T.M.,Frerman F.E.
Kinetic mechanism of glutaryl-CoA dehydrogenase.
Biochemistry
45
15853-15861
2006
1047029
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
1047030
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
1047031
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
1047032
Fu Z.,Wang M.,Paschke R.,Rao K.S.,Frerman F.E.,Kim J.-J.P.
Crystal structures of human glutaryl-CoA dehydrogenase with and without an alternate substrate: structural bases of dehydrogenation and decarboxylation reactions.
Biochemistry
43
9674-9684
2004
1047033
Rao K.S.,Fu Z.,Albro M.,Narayanan B.,Baddam S.,Lee H.-J.K.,Kim J.-J.P.,Frerman F.E.
The effect of a Glu370Asp mutation in glutaryl-CoA dehydrogenase on proton transfer to the dienolate intermediate.
Biochemistry
46
14468-14477
2007
1047034
Biery B.J.,Stein D.E.,Morton D.H.,Goodman S.I.
Gene structure and mutations of glutaryl-coenzyme A dehydrogenase: impaired association of enzyme subunits that is due to an A421V substitution causes glutaric acidemia type I in the Amish.
Am. J. Hum. Genet.
59
1006-1011
1996
1047035
Anikster Y.,Shaag A.,Joseph A.,Mandel H.,Ben-Zeev B.,Christensen E.,Elpeleg O.N.
Glutaric aciduria type I in the Arab and Jewish communities in Israel.
Am. J. Hum. Genet.
59
1012-1018
1996
1047036
Muehlhausen C.,Christensen E.,Schwartz M.,Muschol N.,Ullrich K.,Lukacs Z.
Severe phenotype despite high residual glutaryl-CoA dehydrogenase activity: a novel mutation in a Turkish patient with glutaric aciduria type I.
J. Inherit. Metab. Dis.
26
713-714
2003
1047037
Keyser B.,Muehlhausen C.,Dickmanns A.,Christensen E.,Muschol N.,Ullrich K.,Braulke T.
Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH).
Hum. Mol. Genet.
17
3854-3863
2008
1047038
Georgiou T.,Nicolaidou P.,Hadjichristou A.,Ioannou R.,Dionysiou M.,Siama E.,Chappa G.,Anastasiadou V.,Drousiotou A.
Molecular analysis of Cypriot patients with Glutaric aciduria type I: Identification of two novel mutations.
Clin. Biochem.
47
1300-1305
2014