Reference on EC 1.3.8.7 - medium-chain acyl-CoA dehydrogenase
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REF. 
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
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
Finocchiaro, G.; Ito, M.; Tanaka, K.
Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver
J. Biol. Chem.
262
7982-7989
1987
Homo sapiens
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.; Tanaka, K.
Selective inactivation of various acyl-CoA dehydrogenases by (methylenecyclopropyl)acetyl-CoA
Biochim. Biophys. Acta
1038
216-221
1990
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
Ghisla, S.; Thorpe, C.; Massey, V.
Mechanistic studies with general acyl-CoA dehydrogenase and butyryl-CoA dehydrogenase: evidence for the transfer of the beta-hydrogen to the flavin N(5)-position as a hydride
Biochemistry
23
3154-3161
1984
Sus scrofa
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
Davidson, B.; Schulz, H.
Separation, properties, and regulation of acyl coenzyme A dehydrogenases from bovine heart and liver
Arch. Biochem. Biophys.
213
155-162
1982
Bos taurus
Baldwin, J.E.; Ostrander, R.L.; Simon, C.D.; Widdison, W.C.
Stereospecific inactivation of the general acyl-CoA dehydrogenase from pig kidney by (R)-(-)-(methylenecyclopropyl)acetyl-CoA and (S)-(+)-(methylenecyclopropyl)acetyl-CoA
J. Am. Chem. Soc.
112
2021-2022
1990
Sus scrofa
-
Lenn, N.D.; Stankovich, M.T.; Liu, H.W.
Regulation of the redox potential of general acyl-CoA dehydrogenase by substrate binding
Biochemistry
29
3709-3715
1990
Sus scrofa
Lehmann, T.C.; Hale, D.E.; Bhala, A.; Thorpe, C.
An acyl-coenzyme A dehydrogenase assay utilizing the ferricenium ion
Anal. Biochem.
186
280-284
1990
Sus scrofa
Lai, M.; Liu, H.
Inactivation of general acyl-CoA dehydrogenase by enatiomerically pure (methylencyclopropyl)acetyl-CoA and its implication for this enzyme-catalyzed reaction
J. Am. Chem. Soc.
112
4034-4035
1990
Sus scrofa
-
Zhou, J.Z.; Thorpe, C.
Inactivation of two-electron reduced medium chain acyl-CoA dehydrogenase by 2-octynoyl-CoA
Arch. Biochem. Biophys.
271
261-269
1989
Sus scrofa
Kim, J.J.P.; Wu, J.
Structure of the medium-chain acyl-CoA dehydrogenase from pig liver mitochondria at 3-A resolution
Proc. Natl. Acad. Sci. USA
85
6677-6681
1988
Sus scrofa
Powell, P.J.; Thorpe, C.
2-Octynoyl coenzyme A is a mechanism-based inhibitor of pig kidney medium-chain acyl coenzyme A dehydrogenase: isolation of the target peptide
Biochemistry
27
8022-8028
1988
Sus scrofa
Schopfer, L.M.; Massey, V.; Ghisla, S.; Thorpe, C.
Oxidation-reduction of general acyl-CoA dehydrogenase by the butyryl-CoA/crotonyl-CoA couple. A new investigation of the rapid reaction kinetics
Biochemistry
27
6599-6611
1988
Sus scrofa
Lau, S.M.; Brantley, R.K.; Thorpe, C.
The reductive half-reaction in Acyl-CoA dehydrogenase from pig kidney: studies with thiaoctanoyl-CoA and oxaoctanoyl-CoA analogues
Biochemistry
27
5089-5095
1988
Sus scrofa
Lau, S.M.; Thorpe, C.
The nature of enzyme-substrate complexes in acyl-coenzyme A dehydrogenases
Arch. Biochem. Biophys.
262
293-297
1988
Sus scrofa
Frerman, F.E.
Acyl-CoA dehydrogenases, electron transfer flavoprotein and electron transfer flavoprotein dehydrogenase
Biochem. Soc. Trans.
16
416-418
1988
Mammalia
Matsubara, Y.; Kraus, J.P.; Ozasa, H.; Glassberg, R.; Finocchiaro, G.; Ikeda, Y.; Mole, J.; Rosenberg, L.E.; Tanaka, K.
Molecular cloning and nucleotide sequence of cDNA encoding the entire precursor of rat liver medium chain acyl coenzyme A dehydrogenase
J. Biol. Chem.
262
10104-10108
1987
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
-
Powell, P.J.; Lau, S.M.; Killian, D.; Thorpe, C.
Interaction of acyl coenzyme A substrates and analogues with pig kidney medium-chain acyl-coA dehydrogenase
Biochemistry
26
3704-3710
1987
Sus scrofa
Matsubara, Y.; Kraus, J.P.; Yang-Feng, T.L.; Francke, U.; Rosenberg, L.E.; Tanaka, K.
Molecular cloning of cDNAs encoding rat and human medium-chain acyl-CoA dehydrogenase and assignment of the gene to human chromosome 1
Proc. Natl. Acad. Sci. USA
83
6543-6547
1986
Homo sapiens
Lau, S.M.; Powell, P.; Buettner, H.; Ghisla, S.; Thorpe, C.
Medium-chain acyl coenzyme A dehydrogenase from pig kidney has intrinsic enoyl coenzyme A hydratase activity
Biochemistry
25
4184-4189
1986
Sus scrofa
O'Connell, M.; Henry, S.; Shapiro, L.
Fatty acid degradation in Caulobacter crescentus
J. Bacteriol.
168
49-54
1986
Caulobacter vibrioides
Gustafson, W.G.; Feinberg, B.A.; McFarland, J.T.
Energetics of beta-oxidation. Reduction potentials of general fatty acyl-CoA dehydrogenase, electron transfer flavoprotein, and fatty acyl-CoA substrates
J. Biol. Chem.
261
7733-7741
1986
Sus scrofa
Rojas, C.; Schmidt, J.; Lee, M.Y.; Gustafson, W.G.; McFarland, J.T.
Structure-function correlation of fatty acyl-CoA dehydrogenase and fatty acyl-CoA oxidase
Biochemistry
24
2947-2954
1985
Sus scrofa
Wenz, A.; Ghisla, S.; Thorpe, C.
Studies with general acyl-CoA dehydrogenase from pig kidney. Inactivation by a novel type of suicide inhibitor, 3,4-pentadienoyl-CoA
Eur. J. Biochem.
147
553-560
1985
Sus scrofa
Stabenau, H.; Winkler, U.; Sftel, W.
Enzymes of beta-oxidation in different types of algal microbodies
Plant Physiol.
75
531-533
1984
Eremosphaera viridis, Mougeotia sp.
Madden, M.; Lau, S.M.; Thorpe, C.
The influence of oxidation-reduction state on the kinetic stability of pig kidney general acyl-CoA dehydrogenase and other flavoproteins
Biochem. J.
224
577-580
1984
Sus scrofa
Beckmann, J.D.; Frerman, F.E.
The effects of pH, ionic strength, and chemical modifications on the reaction of electron transfer flavoprotein with an acyl coenzyme A dehydrogenase
J. Biol. Chem.
258
7563-7569
1983
Sus scrofa
Ishii, H.; Ishii, S.; Kazama, M.; Suga, T.
Peroxisomal fatty acyl-coenzyme A oxidation in chicken liver
Arch. Biochem. Biophys.
226
484-491
1983
Gallus gallus
Thorpe, C.; Massey, V.
Flavin analogue studies of pig kidney general acyl-CoA dehydrogenase
Biochemistry
22
2972-2978
1983
Sus scrofa
McFarland, J.T.; Lee, M.Y.; Reinsch, J.; Raven, W.
Reactions of beta-(2-furyl)propionyl coenzyme A with general fatty acyl-CoA dehydrogenase
Biochemistry
21
1224-1229
1982
Sus scrofa
Beckmann, J.D.; Frerman, F.E.; McKean, M.C.
Inhibition of general acyl CoA dehydrogenase by electron transfer flavoprotein semiquinone
Biochem. Biophys. Res. Commun.
102
1290-1294
1981
Sus scrofa
Hall, C.L.
Acyl-CoA dehydrogenases from pig liver mitochondria
Methods Enzymol.
71
375-385
1981
Sus scrofa
Thorpe, C.
Acyl-CoA dehydrogenase from pig kidney
Methods Enzymol.
71
366-374
1981
Sus scrofa
Mizzer, J.P.; Thorpe, C.
Stabilization of the red semiquinone form of pig kidney general acyl-CoA dehydrogenase by acyl coenzyme A derivatives
Biochemistry
20
4965-4970
1981
Sus scrofa
Mayer, E.J.; Thorpe, C.
A method for resolution of general acyl-coenzyme A dehydrogenase apoprotein
Anal. Biochem.
116
227-229
1981
Sus scrofa
Frerman, F.E.; Miziorko, H.M.; Beckmann, J.D.
Enzyme-activated inhibitors, alternate substrates, and a dead end inhibitor of the general acyl-CoA dehydrogenase
J. Biol. Chem.
255
11192-11198
1980
Sus scrofa
Frerman, F.E.; Mielke, D.; Huhta, K.
The functional role of carboxyl residues in an acyl-CoA dehydrogenase
J. Biol. Chem.
255
2199-2202
1980
Sus scrofa
Frerman, F.E.; Kim, J.J.P.; Huhta, K.; McKean, M.C.
Properties of the general acyl-CoA dehydrogenase from pig liver
J. Biol. Chem.
255
2195-2198
1980
Sus scrofa
Hall, C.L.
Acyl-CoA dehydrogenases and electron-transferring flavoprotein
Methods Enzymol.
53
502-518
1978
Bos taurus, Sus scrofa
McKean, M.C.; Frerman, F.E.; Mielke, D.M.
General acyl-CoA dehydrogenase from pig liver. Kinetic and binding studies
J. Biol. Chem.
254
2730-2735
1979
Sus scrofa
Thorpe, C.; Matthews, R.G.; Williams, C.H.
Acyl-coenzyme A dehydrogenase from pig kidney. Purification and properties
Biochemistry
18
331-337
1979
Sus scrofa
Engel, P.C.
Fatty acyl-coenzyme A dehydrogenases
Biochem. Soc. Trans.
6
76-80
1978
Mammalia
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
Hall, C.L.; Kamin, H.
The purification and some properties of electron transfer flavoprotein and general fatty acyl coenzyme A dehydrogenase from pig liver mitochondria
J. Biol. Chem.
250
3476-3486
1975
Sus scrofa
Thorpe, C.; Ciardelli, T.L.; Stewart, C.J.; Wieland, T.
Interaction of long-chain acyl-CoA analogs with pig kidney general acyl-CoA dehydrogenase
Eur. J. Biochem.
118
279-282
1981
Sus scrofa
Mizzer, J.P.; Thorpe, C.
An essential methionine in pig kidney general acyl-CoA dehydrogenase
Biochemistry
19
5500-5504
1980
Sus scrofa
Kim, J.J.; Wang, M.; Paschke, R.
Crystal structures of medium-chain acyl-CoA dehydrogenase from pig liver mitochondria with and without substrate
Proc. Natl. Acad. Sci. USA
90
7523-7527
1993
Sus scrofa
Bross, P.; Jensen, T.G.; Andresen, B.S.; Kjeldsen, M.; Nandy, A.; Kolvraa, S.; Ghisla, S.; Rasched, I.; Bolund, L.; Gregersen, N.
Characterization of wild-type human medium-chain acyl-CoA dehydrogenase (MCAD) and mutant enzymes present in MCAD-deficient patients by two-dimensional gel electrophoresis: evidence for post-translational modification of the enzyme
Biochem. Med. Metab. Biol.
52
36-44
1994
Homo sapiens
Peterson, K.L.; Sergienko, E.E.; Wu, Y.; Kumar, N.R.; Strauss, A.W.; Oleson, A.E.; Muhonen, W.W.; Shabb, J.B.; Srivastava, D.K.
Recombinant human liver medium-chain acyl-CoA dehydrogenase: purification, characterization, and the mechanism of interactions with functionally diverse C8-CoA molecules
Biochemistry
34
14942-14953
1995
Homo sapiens, Sus scrofa
Lee, H.J.; Wang, M.; Paschke, R.; Nandy, A.; Ghisla, S.; Kim, J.J.
Crystal structures of the wild type and the Glu376Gly/Thr255Glu mutant of human medium-chain acyl-CoA dehydrogenase: influence of the location of the catalytic base on substrate specificity
Biochemistry
35
12412-12420
1996
Homo sapiens, Sus scrofa
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
Kieweg, V.; Krautle, F.G.; Nandy, A.; Engst, S.; Vock, P.; Abdel-Ghany, A.G.; Bross, P.; Gregersen, N.; Rasched, I.; Strauss, A.; Ghisla, S.
Biochemical characterization of purified, human recombinant Lys304-->Glu medium-chain acyl-CoA dehydrogenase containing the common disease-causing mutation and comparison with the normal enzyme
Eur. J. Biochem.
246
548-556
1997
Homo sapiens, Sus scrofa
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
Qin, L.; Srivastava, D.K.
Energetics of two-step binding of a chromophoric reaction product, trans-3-indoleacryloyl-CoA, to medium-chain acyl-coenzyme-A dehydrogenase
Biochemistry
37
3499-3508
1998
Sus scrofa
Kuchler, B.; Abdel-Ghany, A.G.; Bross, P.; Nandy, A.; Rasched, I.; Ghisla, S.
Biochemical characterization of a variant human medium-chain acyl-CoA dehydrogenase with a disease-associated mutation localized in the active site
Biochem. J.
337
225-230
1999
Homo sapiens
Baltazar, M.F.; Dickinson, F.M.; Ratledge, C.
Oxidation of medium-chain acyl-CoA esters by extracts of Aspergillus niger: enzymology and characterization of intermediates by HPLC
Microbiology
145
271-278
1999
Aspergillus niger
Lamm, T.R.; Kohls, T.D.; Saenger, A.K.; Stankovich, M.T.
Comparison of ligand polarization and enzyme activation in medium- and short-chain acyl-coenzyme A dehydrogenase-novel analog complexes
Arch. Biochem. Biophys.
409
251-261
2003
Sus scrofa
Gopalan, K.V.; Srivastava, D.K.
Beyond the proton abstracting role of Glu-376 in medium-chain acyl-CoA dehydrogenase: influence of Glu-376-->Gln substitution on ligand binding and catalysis
Biochemistry
41
4638-4648
2002
Homo sapiens
Wu, J.; Bell, A.F.; Luo, L.; Stephens, A.W.; Stankovich, M.T.; Tonge, P.J.
Probing hydrogen-bonding interactions in the active site of medium-chain acyl-CoA dehydrogenase using Raman spectroscopy
Biochemistry
42
11846-11856
2003
Sus scrofa
Nasser, I.; Mohsen, A.W.; Jelesarov, I.; Vockley, J.; Macheroux, P.; Ghisla, S.
Thermal unfolding of medium-chain acyl-CoA dehydrogenase and iso(3)valeryl-CoA dehydrogenase: study of the effect of genetic defects on enzyme stability
Biochim. Biophys. Acta
1690
22-32
2004
Homo sapiens
O'Reilly, L.; Bross, P.; Corydon, T.J.; Olpin, S.E.; Hansen, J.; Kenney, J.M.; McCandless, S.E.; Frazier, D.M.; Winter, V.; Gregersen, N.; Engel, P.C.; Andresen, B.S.
The Y42H mutation in medium-chain acyl-CoA dehydrogenase, which is prevalent in babies identified by MS/MS-based newborn screening, is temperature sensitive
Eur. J. Biochem.
271
4053-4063
2004
Rattus norvegicus
Nishina, Y.; Sato, K.; Tamaoki, H.; Tanaka, T.; Setoyama, C.; Miura, R.; Shiga, K.
Molecular mechanism of the drop in the pKa of a substrate analog bound to medium-chain acyl-CoA dehydrogenase: implications for substrate activation
J. Biochem.
134
835-842
2003
Sus scrofa
Katagiri, H.; Asano, T.; Yamada, T.; Aoyama, T.; Fukushima, Y.; Kikuchi, M.; Kodama, T.; Oka, Y.
Acyl-coenzyme A dehydrogenases are localized on GLUT4-containing vesicles via association with insulin-regulated aminopeptidase in a manner dependent on its dileucine motif
Mol. Endocrinol.
16
1049-1059
2002
Mus musculus
Zeng, J.; Li, D.
Expression and purification of His-tagged rat mitochondrial medium-chain acyl-CoA dehydrogenase wild-type and Arg256 mutant proteins
Protein Expr. Purif.
37
472-478
2004
Rattus norvegicus
Froemming, M.K.; Sames, D.
Fluoromorphic Substrates for fatty acid metabolism: Highly sensitive probes for mammalian medium-chain acyl-CoA dehydrogenase
Angew. Chem.
45
637-642
2006
Rattus norvegicus, Sus scrofa
Zeng, J.; Deng, G.; Yu, W.; Li, D.
Inactivation of medium-chain acyl-CoA dehydrogenase by oct-4-en-2-ynoyl-CoA
Bioorg. Med. Chem. Lett.
16
1445-1448
2006
Rattus norvegicus
Ensenauer, R.; He, M.; Willard, J.M.; Goetzman, E.S.; Corydon, T.J.; Vandahl, B.B.; Mohsen, A.W.; Isaya, G.; Vockley, J.
Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids
J. Biol. Chem.
280
32309-32316
2005
Homo sapiens
Ruswandi, S.; Kitani, K.; Akimitsu, K.; Tsuge, T.; Shiraishi, T.; Yamamoto, M.
Structural analysis of cosmid clone pcAFT-2 carrying AFT10-1 encoding an acyl-CoA dehydrogenase involved in AF-toxin production in the strawberry pathotype of Alternaria alternata
J. Gen. Plant Pathol.
71
107-116
2005
Alternaria alternata
-
Harrison, F.H.; Harwood, C.S.
The pimFABCDE operon from Rhodopseudomonas palustris mediates dicarboxylic acid degradation and participates in anaerobic benzoate degradation
Microbiology
151
1963-1974
2005
Rhodopseudomonas palustris, Actinoplanes friuliensis
Maegawa, G.H.; Poplawski, N.K.; Andresen, B.S.; Olpin, S.E.; Nie, G.; Clarke, J.T.; Teshima, I.
Interstitial deletion of 1p22.2p31.1 and medium-chain acyl-CoA dehydrogenase deficiency in a patient with global developmental delay
Am. J. Med. Genet. A
146A
1581-1586
2008
Homo sapiens
Nichols, M.J.; Saavedra-Matiz, C.A.; Pass, K.A.; Caggana, M.
Novel mutations causing medium chain acyl-CoA dehydrogenase deficiency: under-representation of the common c.985 A > G mutation in the New York state population
Am. J. Med. Genet. A
146A
610-619
2008
Homo sapiens
Gradinaru, R.; Schowen, R.; Ghisla, S.
Solvent isotope effects in reactions of human medium-chain acyl-CoA dehydrogenase active site mutants
Biochemistry
46
2497-2509
2007
Homo sapiens
Zeng, J.; Liu, Y.; Wu, L.; Li, D.
Mutation of Tyr375 to Lys375 allows medium-chain acyl-CoA dehydrogenase to acquire acyl-CoA oxidase activity
Biochim. Biophys. Acta
1774
1628-1634
2007
Rattus norvegicus
Rennison, J.H.; McElfresh, T.A.; Okere, I.C.; Patel, H.V.; Foster, A.B.; Patel, K.K.; Stoll, M.S.; Minkler, P.E.; Fujioka, H.; Hoit, B.D.; Young, M.E.; Hoppel, C.L.; Chandler, M.P.
Enhanced acyl-CoA dehydrogenase activity is associated with improved mitochondrial and contractile function in heart failure
Cardiovasc. Res.
79
331-340
2008
Rattus norvegicus
Tran, K.; Banerjee, S.; Li, H.; Noorani, H.Z.; Mensinkai, S.; Dooley, K.
Clinical efficacy and cost-effectiveness of newborn screening for medium chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry
Clin. Biochem.
40
235-241
2007
Homo sapiens
Ho, S.; Lukacs, Z.; Hoffmann, G.F.; Lindner, M.; Wetter, T.
Feature construction can improve diagnostic criteria for high-dimensional metabolic data in newborn screening for medium-chain acyl-CoA dehydrogenase deficiency
Clin. Chem.
53
1330-1337
2007
Homo sapiens
Herrema, H.; Derks, T.G.; van Dijk, T.H.; Bloks, V.W.; Gerding, A.; Havinga, R.; Tietge, U.J.; Mueller, M.; Smit, G.P.; Kuipers, F.; Reijngoud, D.J.
Disturbed hepatic carbohydrate management during high metabolic demand in medium-chain acyl-CoA dehydrogenase (MCAD)-deficient mice
Hepatology
47
1894-1904
2008
Mus musculus
Derks, T.G.; Boer, T.S.; van Assen, A.; Bos, T.; Ruiter, J.; Waterham, H.R.; Niezen-Koning, K.E.; Wanders, R.J.; Rondeel, J.M.; Loeber, J.G.; Ten Kate, L.P.; Smit, G.P.; Reijngoud, D.J.
Neonatal screening for medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in the Netherlands: the importance of enzyme analysis to ascertain true MCAD deficiency
J. Inherit. Metab. Dis.
31
88-96
2008
Homo sapiens
Cyriac, J.; Venkatesh, V.; Gupta, C.
A fatal neonatal presentation of medium-chain acyl coenzyme a dehydrogenase deficiency
J. Int. Med. Res.
36
609-610
2008
Homo sapiens
Wilcken, B.; Haas, M.; Joy, P.; Wiley, V.; Chaplin, M.; Black, C.; Fletcher, J.; McGill, J.; Boneh, A.
Outcome of neonatal screening for medium-chain acyl-CoA dehydrogenase deficiency in Australia: a cohort study
Lancet
369
37-42
2007
Homo sapiens
Westermann, C.M.; de Sain-van der Velden, M.G.; van der Kolk, J.H.; Berger, R.; Wijnberg, I.D.; Koeman, J.P.; Wanders, R.J.; Lenstra, J.A.; Testerink, N.; Vaandrager, A.B.; Vianey-Saban, C.; Acquaviva-Bourdain, C.; Dorland, L.
Equine biochemical multiple acyl-CoA dehydrogenase deficiency (MADD) as a cause of rhabdomyolysis
Mol. Genet. Metab.
91
362-369
2007
Equus caballus
Westermann, C.M.; Dorland, L.; Votion, D.M.; de Sain-van der Velden, M.G.; Wijnberg, I.D.; Wanders, R.J.; Spliet, W.G.; Testerink, N.; Berger, R.; Ruiter, J.P.; van der Kolk, J.H.
Acquired multiple acyl-CoA dehydrogenase deficiency in 10 horses with atypical myopathy
Neuromuscul. Disord.
18
355-364
2008
Equus caballus
Hsu, H.W.; Zytkovicz, T.H.; Comeau, A.M.; Strauss, A.W.; Marsden, D.; Shih, V.E.; Grady, G.F.; Eaton, R.B.
Spectrum of medium-chain acyl-CoA dehydrogenase deficiency detected by newborn screening
Pediatrics
121
e1108-e1114
2008
Homo sapiens
Yokoi, K.; Ito, T.; Maeda, Y.; Nakajima, Y.; Ueta, A.; Nomura, T.; Koyama, N.; Kato, I.; Suzuki, S.; Kurono, Y.; Sugiyama, N.; Togari, H.
Acylcarnitine profiles during carnitine loading and fasting tests in a Japanese patient with medium-chain acyl-CoA dehydrogenase deficiency
Tohoku J. Exp. Med.
213
351-359
2007
Homo sapiens
Schuck, P.F.; Ferreira, G.d.a..C.; Tonin, A.M.; Viegas, C.M.; Busanello, E.N.; Moura, A.P.; Zanatta, A.; Klamt, F.; Wajner, M.
Evidence that the major metabolites accumulating in medium-chain acyl-CoA dehydrogenase deficiency disturb mitochondrial energy homeostasis in rat brain
Brain Res.
1296
117-126
2009
Rattus norvegicus
Horn, M.P.; Maeder-Heinemann, G.; Andrey, G.; Largiader, C.R.
Mutation screening of the medium-chain acyl-CoA dehydrogenase (MCAD) and the ornithine transcarbamylase (OTC) genes by multiplex PCR amplification and sequencing
Clin. Chem. Lab. Med.
47
56-59
2009
Homo sapiens
Ferreira, A.C.; Orlandi, M.P.; Oliveira, V.C.; Malta, F.S.; Caxito, F.A.; Gomes, K.B.; Valadares, E.R.; Godard, A.L.
A985G mutation incidence in the medium-chain acyl-CoA dehydrogenase (MCAD) gene in Brazil
Genet. Mol. Res.
8
487-493
2009
Homo sapiens
Nishina, Y.; Sato, K.; Tamaoki, H.; Setoyama, C.; Miura, R.; Shiga, K.
FT-IR spectroscopic studies on the molecular mechanism for substrate specificity/activation of medium-chain Acyl-CoA dehydrogenase
J. Biochem.
146
351-357
2009
Sus scrofa
Purevsuren, J.; Kobayashi, H.; Hasegawa, Y.; Mushimoto, Y.; Li, H.; Fukuda, S.; Shigematsu, Y.; Fukao, T.; Yamaguchi, S.
A novel molecular aspect of Japanese patients with medium-chain acyl-CoA dehydrogenase deficiency (MCADD): c.449-452delCTGA is a common mutation in Japanese patients with MCADD
Mol. Genet. Metab.
96
77-79
2009
Homo sapiens
Goetzman, E.S.
The regulation of acyl-CoA dehydrogenases in adipose tissue by rosiglitazone
Obesity (Silver Spring)
17
196-198
2009
Mus musculus
Zeng, J.; Wu, L.; Zhang, X.; Liu, Y.; Deng, G.; Li, D.
Oct-2-en-4-ynoyl-CoA as a specific inhibitor of acyl-CoA oxidase
Org. Lett.
10
4287-4290
2008
Rattus norvegicus
Sturm, M.; Herebian, D.; Mueller, M.; Laryea, M.D.; Spiekerkoetter, U.
Functional effects of different medium-chain acyl-CoA dehydrogenase genotypes and identification of asymptomatic variants
PLoS ONE
7
e45110
2012
Homo sapiens
Bonito, C.A.; Leandro, P.; Ventura, F.V.; Guedes, R.C.
Insights into medium-chain acyl-CoA dehydrogenase structure by molecular dynamics simulations
Chem. Biol. Drug Des.
88
281-292
2016
Homo sapiens (P11310), Homo sapiens, Sus scrofa (P41367), Sus scrofa
Koster, K.L.; Sturm, M.; Herebian, D.; Smits, S.H.; Spiekerkoetter, U.
Functional studies of 18 heterologously expressed medium-chain acyl-CoA dehydrogenase (MCAD) variants
J. Inherit. Metab. Dis.
37
917-928
2014
Homo sapiens (P11310), Homo sapiens
Guzik, M.W.; Narancic, T.; Ilic-Tomic, T.; Vojnovic, S.; Kenny, S.T.; Casey, W.T.; Duane, G.F.; Casey, E.; Woods, T.; Babu, R.P.; Nikodinovic-Runic, J.; OConnor, K.E.
Identification and characterization of an acyl-CoA dehydrogenase from Pseudomonas putida KT2440 that shows preference towards medium to long chain length fatty acids
Microbiology
160
1760-1771
2014
Pseudomonas putida (Q88K54), Pseudomonas putida (Q88L87), Pseudomonas putida (Q88L96), Pseudomonas putida (Q88LN6), Pseudomonas putida, Pseudomonas putida KT 2240 (Q88K54), Pseudomonas putida KT 2240 (Q88L87), Pseudomonas putida KT 2240 (Q88L96), Pseudomonas putida KT 2240 (Q88LN6)
Touw, C.M.; Smit, G.P.; Niezen-Koning, K.E.; Bosgraaf-de Boer, C.; Gerding, A.; Reijngoud, D.J.; Derks, T.G.
In vitro and in vivo consequences of variant medium-chain acyl-CoA dehydrogenase genotypes
Orphanet J. Rare Dis.
8
43
2013
Homo sapiens
Jank, J.M.; Maier, E.M.; Reiss, D.D.; Haslbeck, M.; Kemter, K.F.; Truger, M.S.; Sommerhoff, C.P.; Ferdinandusse, S.; Wanders, R.J.; Gersting, S.W.; Muntau, A.C.
The domain-specific and temperature-dependent protein misfolding phenotype of variant medium-chain acyl-CoA dehydrogenase
PLoS ONE
9
e93852
2014
Homo sapiens (P11310), Homo sapiens
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