Genomes and Genes
Gene Symbol: Acadm
Description: acyl-CoA dehydrogenase medium chain
Alias: MCAD, medium-chain specific acyl-CoA dehydrogenase, mitochondrial, Acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight-chain, acyl-CoA dehydrogenase, C-4 to C-12 straight chain, acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain, acyl-Coenzyme A dehydrogenase, medium chain, medium-chain acyl-CoA dehydrogenase
- He M, Pei Z, Mohsen A, Watkins P, Murdoch G, Van Veldhoven P, et al. Identification and characterization of new long chain acyl-CoA dehydrogenases. Mol Genet Metab. 2011;102:418-29 pubmed publisher..ACAD9 was most highly expressed in the granular layer, ACAD11 in the white matter, and MCAD in the molecular layer and axons of specific neurons...
- 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. 1987;262:7982-9 pubmed..The pattern of substrate specificity for these enzymes determined using ETF as electron acceptor significantly differed from that determined using PMS. All of them were severely inhibited by (methylenecyclopropyl)acetyl-CoA. ..
- Bross P, Engst S, Strauss A, Kelly D, Rasched I, Ghisla S. Characterization of wild-type and an active site mutant of human medium chain acyl-CoA dehydrogenase after expression in Escherichia coli. J Biol Chem. 1990;265:7116-9 pubmed..Properties of the mutant enzyme suggest that the Glu376----Gln change specifically affects substrate binding. These results prove that Glu376 plays an important role in the initial step of dehydrogenation catalysis. ..
- 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. 2007;1774:1628-34 pubmedMedium-chain acyl-CoA dehydrogenase (MCAD) and acyl-CoA oxidase (ACO) are key enzymes catalyzing the rate-determining step for the beta-oxidation of fatty acids...
- Maier E, Gersting S, Kemter K, Jank J, Reindl M, Messing D, et al. Protein misfolding is the molecular mechanism underlying MCADD identified in newborn screening. Hum Mol Genet. 2009;18:1612-23 pubmed publisher..prevalence and genotypic variability than previously estimated, including numerous novel missense mutations in the ACADM gene...
- Willard J, Vicanek C, Battaile K, Van Veldhoven P, Fauq A, Rozen R, et al. Cloning of a cDNA for short/branched chain acyl-Coenzyme A dehydrogenase from rat and characterization of its tissue expression and substrate specificity. Arch Biochem Biophys. 1996;331:127-33 pubmed..Northern blotting of mRNA shows ubiquitous tissue expression of both the rat and human enzyme. Further study of these enzymes will be helpful in understanding structure/function relationships in this gene family. ..
- Ensenauer R, He M, Willard J, Goetzman E, Corydon T, Vandahl B, et al. Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids. J Biol Chem. 2005;280:32309-16 pubmed..Because of the substrate specificity and abundance of ACAD-9 in brain, we speculate that it may play a role in the turnover of lipid membrane unsaturated fatty acids that are essential for membrane integrity and structure. ..
- Frier B, Jacobs R, Wright D. Interactions between the consumption of a high-fat diet and fasting in the regulation of fatty acid oxidation enzyme gene expression: an evaluation of potential mechanisms. Am J Physiol Regul Integr Comp Physiol. 2011;300:R212-21 pubmed publisher..The consumption of an HFD increased the mRNA expression or protein content of medium-chain acyl-CoA dehydrogenase (MCAD), uncoupling protein-3 (UCP3), and pyruvate dehydrogenase kinase 4 (PDK4) in the fed state...
- Tolwani R, Hamm D, Tian L, Sharer J, Vockley J, Rinaldo P, et al. Medium-chain acyl-CoA dehydrogenase deficiency in gene-targeted mice. PLoS Genet. 2005;1:e23 pubmedMedium-chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common inherited disorder of mitochondrial fatty acid beta-oxidation in humans...
- Schuck P, Ferreira G, Tonin A, Viegas C, Busanello E, Moura A, et al. Evidence that the major metabolites accumulating in medium-chain acyl-CoA dehydrogenase deficiency disturb mitochondrial energy homeostasis in rat brain. Brain Res. 2009;1296:117-26 pubmed publisher..Taken together, these data suggest that OA and DA impair brain mitochondrial energy homeostasis that could underlie at least in part the neuropathology of MCADD. ..
- Pruitt K, Maglott D. RefSeq and LocusLink: NCBI gene-centered resources. Nucleic Acids Res. 2001;29:137-40 pubmed..Additional information about LocusLink and RefSeq is available at http://www.ncbi.nlm.nih.gov/LocusLink/. ..
- McMullin P. A skeletal growth defect in the puma (Felis concolor). Vet Rec. 1978;103:356-8 pubmed..This case may be an example of hereditary chondrodysplastic dwarfism in the puma. An unusual finding was the presence of iron in the cartilage of abnormal growth plates. ..
- Saranteas T, Zotos N, Lolis E, Stranomiti J, Mourouzis C, Chantzi C, et al. Mechanisms of ketamine action on lipid metabolism in rats. Eur J Anaesthesiol. 2005;22:222-6 pubmed..LPL activity in adipose tissue and medium-chain acyl-CoA dehydrogenase (MCAD) content in muscle were determined...
- Kim J, Choi S, Lee H, Hwang I, Lee Y, An B, et al. Selenium significantly inhibits adipocyte hypertrophy and abdominal fat accumulation in OLETF rats via induction of fatty acid ?-oxidation. Biol Trace Elem Res. 2012;150:360-70 pubmed publisher..fat, adiponectin, cholesterol, very long chain dehydrogenase (VLCAD), and medium chain acyl-CoA dehydrogenase (MCAD) in 20-week-old Otsuka Long-Evans Tokushima Fatty (OLETF) rats following sodium selenite treatment for 2 weeks...
- Herrema H, Derks T, van Dijk T, Bloks V, Gerding A, Havinga R, et al. Disturbed hepatic carbohydrate management during high metabolic demand in medium-chain acyl-CoA dehydrogenase (MCAD)-deficient mice. Hepatology. 2008;47:1894-904 pubmed publisherMedium-chain acyl-coenzyme A (CoA) dehydrogenase (MCAD) catalyzes crucial steps in mitochondrial fatty acid oxidation, a process that is of key relevance for maintenance of energy homeostasis, especially during high metabolic demand...
- Schuck P, Ferreira G, Moura A, Busanello E, Tonin A, Dutra Filho C, et al. Medium-chain fatty acids accumulating in MCAD deficiency elicit lipid and protein oxidative damage and decrease non-enzymatic antioxidant defenses in rat brain. Neurochem Int. 2009;54:519-25 pubmed publisher..It is therefore presumed that this pathomechanism may be involved in the pathophysiology of the neurologic symptoms manifested by patients affected by MCADD. ..
- Djouadi F, Riveau B, Merlet Benichou C, Bastin J. Tissue-specific regulation of medium-chain acyl-CoA dehydrogenase gene by thyroid hormones in the developing rat. Biochem J. 1997;324 ( Pt 1):289-94 pubmedDuring development, gene expression of medium-chain acyl-CoA dehydrogenase (MCAD), a nuclear-encoded mitochondrial enzyme that catalyses the first step of medium-chain fatty acid beta-oxidation, is highly regulated in tissues in ..
- Djouadi F, Bastin J, Kelly D, Merlet Benichou C. Transcriptional regulation by glucocorticoids of mitochondrial oxidative enzyme genes in the developing rat kidney. Biochem J. 1996;315 ( Pt 2):555-62 pubmed..We therefore studied the changes in the steady-state levels of mRNA encoding medium-chain acyl-CoA dehydrogenase (MCAD), which catalyses the initial step in mitochondrial fatty acid beta-oxidation, in the rat kidney cortex and medulla ..
- Matsubara Y, Indo Y, Naito E, Ozasa H, Glassberg R, Vockley J, et al. 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 famil. J Biol Chem. 1989;264:16321-31 pubmed..Comparison of the sequences of four rat acyl-CoA dehydrogenases, including LCAD, MCAD, SCAD, and IVD, and two of their human counterparts (MCAD and SCAD) reveals a high degree of homology (57 invariant ..
- MaÅ‚ecki J, Ho A, Moen A, Dahl H, Falnes P. Human METTL20 is a mitochondrial lysine methyltransferase that targets the Î² subunit of electron transfer flavoprotein (ETFÎ²) and modulates its activity. J Biol Chem. 2015;290:423-34 pubmed publisher..Based on the previous naming of similar enzymes, we suggest the renaming of human METTL20 to ETFÎ²-KMT. ..
- Murase T, Aoki M, Tokimitsu I. Supplementation with alpha-linolenic acid-rich diacylglycerol suppresses fatty liver formation accompanied by an up-regulation of beta-oxidation in Zucker fatty rats. Biochim Biophys Acta. 2005;1733:224-31 pubmed..the up-regulation of beta-oxidation activity, and acyl-CoA oxidase (ACO) and medium-chain acyl-CoA dehydrogenase (MCAD) mRNA levels...
- Matsubara Y, Narisawa K, Miyabayashi S, Tada K, Coates P, Bachmann C, et al. Identification of a common mutation in patients with medium-chain acyl-CoA dehydrogenase deficiency. Biochem Biophys Res Commun. 1990;171:498-505 pubmedMedium-chain acyl-CoA dehydrogenase (MCAD) deficiency is one of the most common recessively inherited metabolic diseases in man...
- Huidekoper H, Schneider J, Westphal T, Vaz F, Duran M, Wijburg F. Prolonged moderate-intensity exercise without and with L-carnitine supplementation in patients with MCAD deficiency. J Inherit Metab Dis. 2006;29:631-6 pubmed..Our results suggest that MCADD patients are able to increase carnitine biosynthesis during exercise to compensate for carnitine losses. ..
- Ghisla S, Thorpe C. Acyl-CoA dehydrogenases. A mechanistic overview. Eur J Biochem. 2004;271:494-508 pubmed..These views are also discussed in the context of the accompanying paper on the three-dimensional properties of acyl-CoA dehydrogenases. ..