Reference on EC 1.3.8.8 - long-chain acyl-CoA dehydrogenase
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REF. 
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Suzuki, H.; Yamada, J.; Watanabe, T.; Suga, T.
Compartmentation of dicarboxylic acid beta-oxidation in rat liver: importance of peroxisomes in the metabolism of dicarboxylic acids
Biochim. Biophys. Acta
990
25-30
1989
Rattus norvegicus
Matsubara, Y.; Indo, Y.; Naito, E.; Ozasa, H.; Glassberg, R.; Vockley, J.; Ikeda, Y.; Kraus, J.; Tanaka, K.
Molecular cloning and nucleotide sequence of cDNAs encoding the precursors of rat long chain acyl-coenzyme A, short chain acyl-coenzyme A, and isovaleryl-coenzyme A dehydrogenases. Sequence homology of four enzymes of the acyl-CoA dehydrogenase family
J. Biol. Chem.
264
16321-16331
1989
Rattus norvegicus
Ikeda, Y.; Dabrowski, C.; Tanaka, K.
Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase
J. Biol. Chem.
258
1066-1076
1983
Rattus norvegicus
Ikeda, Y.; Keese, S.M.; Fenton, W.A.; Tanaka, K.
Biosynthesis of four rat liver mitochondrial acyl-CoA dehydrogenases: in vitro synthesis, import into mitochondria, and processing of their precursors in a cell-free system and in cultured cells
Arch. Biochem. Biophys.
252
662-674
1987
Rattus norvegicus
Ikeda, Y.; Okamura-Ikeda, K.; Tanaka, K.
Spectroscopic analysis of the interaction of rat liver short-chain, medium-chain, and long-chain acyl coenzyme A dehydrogenases with acyl coenzyme A substrates
Biochemistry
24
7192-7199
1985
Rattus norvegicus
Okamura-Ikeda, K.; Ikeda, Y.; Tanaka, K.
An essential cysteine residue located in the vicinity of the FAD-binding site in short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria
J. Biol. Chem.
260
1338-1345
1985
Rattus norvegicus
Ikeda, Y.; Hine, D.G.; Okamura-Ikeda, K.; Tanaka, K.
Mechanism of action of short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases. Direct evidence for carbanion formation as an intermediate step using enzyme-catalyzed C-2 proton/deuteron exchange in the absence of C-3 exchange
J. Biol. Chem.
260
1326-1337
1985
Rattus norvegicus
Ikeda, Y.; Okamura-Ikeda, K.; Tanaka, K.
Purification and characterization of short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. Isolation of the holo- and apoenzymes and conversion of the apoenzyme to the holoenzyme
J. Biol. Chem.
260
1311-1325
1985
Rattus norvegicus
Dommes, V.; Kunau, W.H.
Purification and properties of acyl coenzyme A dehydrogenases from bovine liver. Formation of 2-trans,4-cis-decadienoyl coenzyme A
J. Biol. Chem.
259
1789-1797
1984
Bos taurus
Furuta, S.; Miyazawa, S.; Hashimoto, T.
Purification and properties of rat liver acyl-CoA dehydrogenases and electron transfer flavoprotein
J. Biochem.
90
1739-1750
1981
Rattus norvegicus
Veitch, K.; Draye, J.P.; van Hoof, F.; Stanley, H.; Sherratt, H.S.
Effects of riboflavin deficiency and clofibrate treatment on the five acyl-CoA dehydrogenases in rat liver mitochondria
Biochem. J.
254
477-481
1988
Rattus norvegicus
Chalmers, R.A.; English, N.
Fatty acid oxidation in cultured skin fibroblasts from patients with inherited disorders of fatty acid metabolism: studies on the specificity of acyl-CoA dehydrogenases
Biochem. Soc. Trans.
15
489-491
1987
Homo sapiens
-
Hall, C.L.; Heijkenskjld, L.; Bartfai, T.; Ernster, L.; Kamin, H.
Acyl coenzyme A dehydrogenases and electron-transferring flavoprotein from beef hart mitochondria
Arch. Biochem. Biophys.
177
402-414
1976
Bos taurus
Nandy, A.; Kieweg, V.; Kraeutle, F.G.; Vock, P.; Kuechler, B.; Bross, P.; Kim, J.J.P.; Rasched, I.; Ghisla, S.
Medium-long-chain chimeric human acyl-CoA dehydrogenase: medium-chain enzyme with the active center base arrangement of long-chain acyl-CoA dehydrogenase
Biochemistry
35
12402-12411
1996
Homo sapiens
Eder, M.; Krautle, F.; Dong, Y.; Vock, P.; Kieweg, V.; Kim, J.J.; Strauss, A.W.; Ghisla, S.
Characterization of human and pig kidney long-chain-acyl-CoA dehydrogenases and their role in beta-oxidation
Eur. J. Biochem.
245
600-607
1997
Homo sapiens, Sus scrofa
Yamada, J.; Ogawa, S.; Horie, S.; Watanabe, T.; Suga, T.
Participation of peroxisomes in the metabolism of xenobiotic acyl compounds: comparison between peroxisomal and mitochondrial beta-oxidation of omega-phenyl fatty acids in rat liver
Biochim. Biophys. Acta
921
292-301
1987
Rattus norvegicus
Hauge, J.G.; Crane, F.L.; Beinert, H.
On the mechanism of dehydrogenation of fatty acyl derivatives of coenzyme A
J. Biol. Chem.
219
727-733
1956
Sus scrofa
Bauche, F.; Sabourault, D.; Giudicelli, Y.; Nordmann, J.; Nordmann, R.
2-Mercaptoacetate administration depresses the beta-oxidation pathway through an inhibition of long-chain acyl-CoA dehydrogenase activity
Biochem. J.
196
803-809
1981
Rattus norvegicus
Gohil, K.; Jones, D.A.; Edwards, R.H.T.
Fatty acid oxidation in human skeletal muscle mitochondria
Biochem. Soc. Trans.
11
728-729
1983
Homo sapiens
-
Furuta, S.; Miyazawa, S.; Hashimoto, T.
Induction of acyl-CoA dehydrogenases and electron transfer flavoprotein and their roles in fatty acid oxidation in rat liver mitochondria
J. Biochem.
90
1751-1756
1981
Rattus norvegicus
Hughes, K.T.; Simons R.W.; Nunn, W.D.
Regulation of fatty acid degradation in Escherichia coli: fadR superrepressor mutants are unable to utilize fatty acids as the sole carbon source
J. Bacteriol.
170
1666-1671
1988
Escherichia coli
Veitch, K.; Draye, J.P.; Vamecq, J.; Causey, A.G.; Barlett, K.; Sherratt, H.S.A; Van Hoof,F.
Altered acyl-CoA metabolism in riboflavin deficiency
Biochim. Biophys. Acta
1006
335-343
1989
Rattus norvegicus
Ijlst, L.; Wanders,R.J.A.
A simple spectrophotometric assay for long-chain acyl-CoA dehydrogenase activity measurements in human skin fibroblasts
Ann. Clin. Biochem.
30
293-297
1993
Homo sapiens
-
Djordjevic, S.; Dong, Y.; Paschke, R.; Frerman, F.E.; Strauss, A.W.; Kim, J.J.
Identification of the catalytic base in long chain acyl-CoA dehydrogenase
Biochemistry
33
4258-4264
1994
Homo sapiens
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
Homo sapiens, Rattus norvegicus
Battaile, K.P.; McBurney, M.; Van Veldhoven, P.P.; Vockley, J.
Human long chain, very long chain and medium chain acyl-CoA dehydrogenases are specific for the S-enantiomer of 2-methylpentadecanoyl-CoA
Biochim. Biophys. Acta
1390
333-338
1998
Homo sapiens
Kurtz, D.M.; Tolwani, R.J.; Wood, P.A.
Structural characterization of the mouse long-chain acyl-CoA dehydrogenase gene and 5' regulatory region
Mamm. Genome
9
361-365
1998
Mus musculus (P51174), Mus musculus
Bode, K.; Hooks, M.A.; Couee, I.
Identification, separation, and characterization of acyl-coenzyme A dehydrogenases involved in mitochondrial beta-oxidation in higher plants
Plant Physiol.
119
1305-1314
1999
Helianthus annuus
Takagi, M.; Yamakawa, H.; Watanabe, T.; Suga, T.; Yamada, J.
Inducible expression of long-chain acyl-CoA hydrolase gene in cell cultures
Mol. Cell. Biochem.
252
379-385
2003
Mus musculus
Tani, A.; Ishige, T.; Sakai, Y.; Kato, N.
Two acyl-CoA dehydrogenases of Acinetobacter sp. strain M-1 that uses very long-chain n-alkanes
J. Biosci. Bioeng.
94
326-329
2002
Acinetobacter sp. (Q8L0X1), Acinetobacter sp., Acinetobacter sp. M-1 (Q8L0X1)
Clark-Taylor, T.; Clark-Taylor, B.E.
Is autism a disorder of fatty acid metabolism? Possible dysfunction of mitochondrial beta-oxidation by long chain acyl-CoA dehydrogenase
Med. Hypotheses
62
970-975
2004
Homo sapiens
Berger, P.S.; Wood, P.A.
Disrupted blastocoele formation reveals a critical developmental role for long-chain acyl-CoA dehydrogenase
Mol. Genet. Metab.
82
266-272
2004
Mus musculus
Takagi, M.; Ohtomo, T.; Hiratsuka, K.; Kuramochi, Y.; Suga, T.; Yamada, J.
Localization of a long-chain acyl-CoA hydrolase in spermatogenic cells in mice
Arch. Biochem. Biophys.
446
161-166
2006
Mus musculus
Yamada, J.
Long-chain acyl-CoA hydrolase in the brain
Amino Acids
28
273-278
2005
Homo sapiens, Mus musculus, Rattus norvegicus
Mason, K.E.; Stofan, D.A.; Szweda, L.I.
Inhibition of very long chain acyl-CoA dehydrogenase during cardiac ischemia
Arch. Biochem. Biophys.
437
138-143
2005
Rattus norvegicus
Oey, N.A.; Ruiter, J.P.N.; Ijlst, L.; Attie-Bitach, T.; Vekemans, M.; Wanders, R.J.A.; Wijburg, F.A.
Acyl-CoA dehydrogenase 9 (ACAD 9) is the long-chain acyl-CoA dehydrogenase in human embryonic and fetal brain
Biochem. Biophys. Res. Commun.
346
33-37
2006
Homo sapiens
Djouadi, F.; Aubey, F.; Schlemmer, D.; Ruiter, J.P.N.; Wanders, R.J.A.; Strauss, A.W.; Bastin, J.
Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders
Hum. Mol. Genet.
14
2695-2703
2005
Homo sapiens
Merritt II, J.L.; Matern, D.; Vockley, J.; Daniels, J.; Ngyen, T.V.; Schowalter, D.B.
In vitro characterization and in vivo expression of human very-long chain acyl-CoA dehydrogenase
Mol. Genet. Metab.
88
351-358
2006
Homo sapiens
Gobin-Limballe, S.; Djouadi, F.; Aubey, F.; Olpin, S.; Andresen, B.S.; Yamaguchi, S.; Mandel, H.; Fukao, T.; Ruiter, J.P.; Wanders, R.J.; McAndrew, R.; Kim, J.J.; Bastin, J.
Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapy
Am. J. Hum. Genet.
81
1133-1143
2007
Homo sapiens (P49748), Homo sapiens
Werdich, A.A.; Baudenbacher, F.; Dzhura, I.; Jeyakumar, L.H.; Kannankeril, P.J.; Fleischer, S.; LeGrone, A.; Milatovic, D.; Aschner, M.; Strauss, A.W.; Anderson, M.E.; Exil, V.J.
Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice
Am. J. Physiol. Heart Circ. Physiol.
292
H2202-H2211
2007
Mus musculus
Law, L.K.; Tang, N.L.; Tong, M.K.; Mak, T.W.; Wanders, R.J.; Lam, C.W.
Novel missense mutations in the first Chinese patient with very-long-chain acyl-CoA dehydrogenase deficiency
Clin. Chim. Acta
375
173-174
2007
Homo sapiens (P49748)
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
Homo sapiens
Zia, A.; Kolodny, E.H.; Pastores, G.M.
Very long chain acyl-CoA dehydrogenase deficiency in a pair of mildly affected monozygotic twin sister in their late fifties
J. Inherit. Metab. Dis.
30
817-817
2007
Homo sapiens
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
Homo sapiens
Primassin, S.; Ter Veld, F.; Mayatepek, E.; Spiekerkoetter, U.
Carnitine supplementation induces acylcarnitine production in tissues of very long-chain acyl-CoA dehydrogenase-deficient mice, without replenishing low free carnitine
Pediatr. Res.
63
632-637
2008
Mus musculus
Tajima, G.; Sakura, N.; Shirao, K.; Okada, S.; Tsumura, M.; Nishimura, Y.; Ono, H.; Hasegawa, Y.; Hata, I.; Naito, E.; Yamaguchi, S.; Shigematsu, Y.; Kobayashi, M.
Development of a new enzymatic diagnosis method for very-long-chain acyl-CoA dehydrogenase deficiency by detecting 2-hexadecenoyl-CoA production and its application in tandem mass spectrometry-based selective screening and newborn screening in Japan
Pediatr. Res.
64
667-672
2008
Homo sapiens
Zhang, D.; Liu, Z.X.; Choi, C.S.; Tian, L.; Kibbey, R.; Dong, J.; Cline, G.W.; Wood, P.A.; Shulman, G.I.
Mitochondrial dysfunction due to long-chain acyl-CoA dehydrogenase deficiency causes hepatic steatosis and hepatic insulin resistance
Proc. Natl. Acad. Sci. USA
104
17075-17080
2007
Mus musculus
Beattie, S.G.; Goetzman, E.; Tang, Q.; Conlon, T.; Campbell-Thompson, M.; Matern, D.; Vockley, J.; Flotte, T.R.
Recombinant adeno-associated virus-mediated gene delivery of long chain acyl coenzyme A dehydrogenase (LCAD) into LCAD-deficient mice
J. Gene Med.
10
1113-1123
2008
Mus musculus
Arnold, G.L.; Van Hove, J.; Freedenberg, D.; Strauss, A.; Longo, N.; Burton, B.; Garganta, C.; Ficicioglu, C.; Cederbaum, S.; Harding, C.; Boles, R.G.; Matern, D.; Chakraborty, P.; Feigenbaum, A.
A Delphi clinical practice protocol for the management of very long chain acyl-CoA dehydrogenase deficiency
Mol. Genet. Metab.
96
85-90
2009
Homo sapiens
Korematsu, S.; Kosugi, Y.; Kumamoto, T.; Yamaguchi, S.; Izumi, T.
Novel mutation of early, perinatal-onset, myopathic-type very-long-chain acyl-CoA dehydrogenase deficiency
Pediatr. Neurol.
41
151-153
2009
Homo sapiens
ter Veld, F.; Mueller, M.; Kramer, S.; Haussmann, U.; Herebian, D.; Mayatepek, E.; Laryea, M.D.; Primassin, S.; Spiekerkoetter, U.
A novel tandem mass spectrometry method for rapid confirmation of medium- and very long-chain acyl-CoA dehydrogenase deficiency in newborns
PLoS ONE
4
e6449
2009
Homo sapiens
Li, Z.; Zhai, Y.; Fang, J.; Zhou, Q.; Geng, Y.; Sun, F.
Purification, crystallization and preliminary crystallographic analysis of very-long-chain acyl-CoA dehydrogenase from Caenorhabditis elegans
Acta Crystallogr. Sect. F
66
426-430
2010
Caenorhabditis elegans
Maher, A.C.; Mohsen, A.W.; Vockley, J.; Tarnopolsky, M.A.
Low expression of long-chain acyl-CoA dehydrogenase in human skeletal muscle
Mol. Genet. Metab.
100
163-167
2010
Homo sapiens
Rudolf, J.D.; Dong, L.B.; Huang, T.; Shen, B.
A genetically amenable platensimycin- and platencin-overproducer as a platform for biosynthetic explorations: a showcase of PtmO4, a long-chain acyl-CoA dehydrogenase
Mol. Biosyst.
11
2717-2726
2015
Streptomyces platensis (D8L2U8), Streptomyces platensis SB12029 (D8L2U8), Streptomyces platensis SB12029
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