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ACADSB  -  acyl-CoA dehydrogenase, short/branched chain

Homo sapiens

Synonyms: 2-MEBCAD, 2-methyl branched chain acyl-CoA dehydrogenase, 2-methylbutyryl-CoA dehydrogenase, 2-methylbutyryl-coenzyme A dehydrogenase, ACAD7, ...
 
 
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Disease relevance of ACADSB

 

High impact information on ACADSB

 

Biological context of ACADSB

 

Anatomical context of ACADSB

 

Associations of ACADSB with chemical compounds

  • 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: a new inborn error of L-isoleucine metabolism [6].
  • Residues Met-361 and Ala-365 of 2MBCD appear to partially substitute for the function of Tyr-380 in human SBCAD, binding the methyl branch linked to C2 of 2-methylbutyryl-CoA, whereas residues Val-88, Val-92, and Val-96 appear to bind the distal C4 methyl group [4].
  • Tandem MS analysis of acylcarnitines may reveal elevated C5 (SBCAD) or C5:1 and/or OH-C5 species (MHBD and beta-KT deficiencies) but the abnormalities are non-diagnostic and may be intermittent or absent [2].
  • These results and those of other studies suggest that testosterone controls the enzymes isovaleryl acyl-CoA dehydrogenase and 2-methyl branched-chain acyl-CoA dehydrogenase; in the absence of these enzymes, the primers for the synthesis of methyl-branched chain fatty acids are produced [8].
  • The enzymatic basis for the metabolism and inhibitory effects of valproic acid: dehydrogenation of valproyl-CoA by 2-methyl-branched-chain acyl-CoA dehydrogenase [9].
 

Other interactions of ACADSB

  • Sequence analysis of candidate ACADs revealed heterozygosity for the common short-chain ACAD A625 variant allele and no mutations in ACAD-8 but a 100-bp deletion in short/branched-chain ACAD (SBCAD) cDNA from the patient [3].

References

  1. Association of genetic polymorphisms of ACADSB and COMT with human hypertension. Kamide, K., Kokubo, Y., Yang, J., Matayoshi, T., Inamoto, N., Takiuchi, S., Horio, T., Miwa, Y., Yoshii, M., Tomoike, H., Tanaka, C., Banno, M., Okuda, T., Kawano, Y., Miyata, T. J. Hypertens. (2007) [Pubmed]
  2. Inborn errors of isoleucine degradation: A review. Korman, S.H. Mol. Genet. Metab. (2006) [Pubmed]
  3. Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism. Andresen, B.S., Christensen, E., Corydon, T.J., Bross, P., Pilgaard, B., Wanders, R.J., Ruiter, J.P., Simonsen, H., Winter, V., Knudsen, I., Schroeder, L.D., Gregersen, N., Skovby, F. Am. J. Hum. Genet. (2000) [Pubmed]
  4. Convergent evolution of a 2-methylbutyryl-CoA dehydrogenase from isovaleryl-CoA dehydrogenase in Solanum tuberosum. Goetzman, E.S., Mohsen, A.W., Prasad, K., Vockley, J. J. Biol. Chem. (2005) [Pubmed]
  5. Localization of short/branched chain acyl-CoA dehydrogenase (ACADSB) to human chromosome 10. Arden, K.C., Viars, C.S., Fu, K., Rozen, R. Genomics (1995) [Pubmed]
  6. 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: a new inborn error of L-isoleucine metabolism. Gibson, K.M., Burlingame, T.G., Hogema, B., Jakobs, C., Schutgens, R.B., Millington, D., Roe, C.R., Roe, D.S., Sweetman, L., Steiner, R.D., Linck, L., Pohowalla, P., Sacks, M., Kiss, D., Rinaldo, P., Vockley, J. Pediatr. Res. (2000) [Pubmed]
  7. Androgenic control of 1-alkyl-2,3-diacylglycerol in the harderian gland of the golden hamster, Mesocricetus auratus. Seyama, Y., Hida, A., Hayashi, S., Buzzell, G.R. J. Biochem. (1996) [Pubmed]
  8. Effect of the photoperiod in modulating the androgenic control of 1-alkyl-2,3-diacylglycerol composition in the harderian gland of the golden hamster, Mesocricetus auratus. Buzzell, G.R., Hida, A., Fu, S., Seyama, Y. J. Exp. Zool. (1997) [Pubmed]
  9. The enzymatic basis for the metabolism and inhibitory effects of valproic acid: dehydrogenation of valproyl-CoA by 2-methyl-branched-chain acyl-CoA dehydrogenase. Ito, M., Ikeda, Y., Arnez, J.G., Finocchiaro, G., Tanaka, K. Biochim. Biophys. Acta (1990) [Pubmed]
 
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