Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver.
نویسندگان
چکیده
منابع مشابه
Acyl-CoA dehydrogenases and acyl-CoA oxidases
Acyl-CoA dehydrogenases and acyl-CoA oxidases are two closely related FAD-containing enzyme families that are present inmitochondria andperoxisomes, respectively. They catalyze the dehydrogenation of acyl-CoA thioesters to the corresponding trans-2-enoyl-CoA. This review examines the structure of medium chain acyl-CoA dehydrogenase, as a representative of the dehydrogenase family, with respect ...
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 ...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 1987
ISSN: 0021-9258
DOI: 10.1016/s0021-9258(18)47514-x