Molecular evolution and substrate specificity of acyl-CoA dehydrogenases: Chimaeric ‘medium/long’ chain-specific enzyme from medium-chain acyl-CoA dehydrogenase
نویسندگان
چکیده
منابع مشابه
Molecular evolution and substrate specificity of acyl-CoA dehydrogenases: chimaeric "medium/long' chain-specific enzyme from medium-chain acyl-CoA dehydrogenase.
5385-5392 37 Enemark, J. H. and Young, C. G. (1993) Adv. Inorg. Chem. 40, 1-88 37a Howes, B. D., Bray, R. C., Richards, R. LA., Turner, N. A., Bennett, B. and Lowe, D. J. (1996) Biochemistry, in the press 38 Howes, B. D., Bennett, B., Bray, R. C., Richards, R. L. and Lowe, D. J. (1994) J. Am. Chem. Soc. 116, 11624-1 1625 39 Gambarotta, S., Floriani, C., Chiesi-Villa, A. and Guastini, C. (1985) ...
متن کاملAcyl-CoA Dehydrogenases: Mechanistic studies on Medium Chain Acyl-CoA Dehydrogenase
ion of Glu99, a group with potential base functionality that is also present at the “bottom” of the active center (Kim et al., 1993); b) to gain information on whether the activity observed with the Glu376Gln mutant is specific to the 376Gln amide group itself. The pH dependence of the enzyme flavin reduction rates for these mutants was measured as described above for Glu376Gln-MCAD under anaer...
متن کامل[Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency].
common disorder of fatty acid oxidation affecting 1 in 13,000 newborns and is inherited as an autosomal recessive disorder. This enzyme deficiency results in the inability to catabolize medium-chain (6-12 carbon molecules) fatty acids for energy utilization. MCAD deficiency often presents in the first two years of life after viral illness or fasting. This inability to break down medium-chain li...
متن کامل[Medium-chain acyl-CoA dehydrogenase deficiency].
From 65 reported cases of medium chain acyl-CoA dehydrogenase deficiency, we found an average presenting age of 13.5 months and a mean age at death of 18.5 months. One quarter of patients died of a Reye-like syndrome and/or sudden infant death. In half the cases there had been at least one sibling death. Asymptomatic cases were not uncommon (12% of cases). The crises were generally induced by a...
متن کاملMedium chain acyl-CoA dehydrogenase deficiency
From 65 reported cases of medium chain acylCoA dehydrogenase deficiency, we found an average presenting age of 13-5 months and a mean age at death of 18-5 months. One quarter of patients died of a Reye-like syndrome and/or sudden infant death. In half the cases there had been at least one sibling death. Asymptomatic cases were not uncommon (12% of cases). The crises were generally induced by a ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Biochemical Society Transactions
سال: 1996
ISSN: 0300-5127,1470-8752
DOI: 10.1042/bst0240105