منابع مشابه
Fatty Acid oxidation defects.
Fatty acid oxidation defects (FAODs) are a group of inborn errors of metabolism that carry a risk for morbidity and mortality. Successful management of these conditions involves early diagnosis, proper management of clinical situations that predisposes to metabolic crisis as well as adequate treatment of a metabolic crisis. The advent of tandem mass spectrometry based newborn screening has redu...
متن کاملFatty acid oxidation
Fatty acids are the preferred oxidative substrates of the heart, skeletal muscles, kidney cortex and liver in adult mammals. They are supplied to these tissues either as nonesterified fatty acids (NEFAI, or as triglycerides after hydrolysis by lipoprotein lipase. During fetal life, tissue capacity to oxidize NEFA is very low, even in species in which the placental transfer of NEFA and carnitine...
متن کاملFatty Acid Oxidation
The heart consumes vast amounts of energy, and cardiac energetic impairment is a characteristic feature of several inherited and acquired diseases of heart muscle. In the fasting state, the heart typically generates ≈70% of its energy from β-oxidation of fatty acids, 10% to 30% from oxidation of glucose, and the remainder from ketones, lactate, and amino acids. However, it is a metabolic omnivo...
متن کاملStudies of fatty acid oxidation. 3. The effects of acyl-CoA complexes on fatty acid oxidation.
The oxidation of fatty acids to acetoacetate or to carbon dioxide is affected by the presence of various aliphatic and aromatic acids. For example, benzoate inhibits acetoacetate formation from butyrate at low concentrations in guinea pig liver slices (Jowett & Quastel, 1935b) and in 'cyclophorase' preparations (Grafflin & Green, 1948). Benzoate also inhibits the oxidation of butyrate to C02 in...
متن کاملProcessivity and fatty acid oxidation.
446 TDRAs, through the partial and group response coefficients, confirm that, under every condition that we have studied, malonyl-CoA exerts its effects over the pathway via CPT I. These conclusions apply only to our system and our conditions. Yet our TDCAs in a more physiological system, including ketone body and ATP synthesis in coupled mitochondria, show that in state 3 CPT I has most of the...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 1944
ISSN: 0021-9258
DOI: 10.1016/s0021-9258(18)72012-7