منابع مشابه
Analysis of fatty acid oxidation intermediates in cultured fibroblasts to detect mitochondrial oxidation disorders.
We describe a method for the diagnosis of mitochondrial fatty acid oxidation disorders that is based on the analysis of acylcarnitine and acyl-coenzyme A (acyl-CoA) esters generated during fatty acid oxidation by permeabilized skin fibroblasts. This method requires only small amounts of cultured fibroblasts with minimal preparation, and no isolation of mitochondrial fractions is necessary. Duri...
متن کاملInherited defects of mitochondrial fatty acid oxidation.
soma1 oxidase ( H 2 0 , production). Nevertheless, mitochondrial oxidation of sebacyl-carnitine could be recorded. This activity was 10-20% of that measured in the same mitochondrial preparation with palmitoyl-CoA ( + L-carnitine) or decanoyl-carnitine. Further, there was no indication of interference by sebacyl-CoA with carnitine palmitoyltransferase. In conclusion, intravenously infused dodec...
متن کاملMitochondrial fatty acid oxidation in obesity.
SIGNIFICANCE Current lifestyles with high-energy diets and little exercise are triggering an alarming growth in obesity. Excess of adiposity is leading to severe increases in associated pathologies, such as insulin resistance, type 2 diabetes, atherosclerosis, cancer, arthritis, asthma, and hypertension. This, together with the lack of efficient obesity drugs, is the driving force behind much r...
متن کاملPrenatal diagnosis of disorders of fatty acid transport and mitochondrial oxidation.
Fatty acid transport and mitochondrial oxidation (FATMO) is a complex pathway that plays a major role in energy production during fasting or when illness and stressful situations require higher energy consumption. After long-chain fatty acids are mobilized from adipose tissue, taken up by liver and muscle cells, and activated to coenzyme A esters, they are transported into mitochondria by the s...
متن کاملLCHAD and MTP Deficiencies - Two Disorders of Mitochondrial Fatty Acid -Oxidation with Unusual Features
Mitochondrial fatty acid -oxidation disorders are relatively common causes of acute metabolic crises and sudden death in infants. Most of these disorders can be treated effectively, provided, fasting is avoided and there is an early start of a high-carbohydrate low-fat diet therapy. Two disorders, long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) and complete mitochondrial trifunctional protei...
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ژورنال
عنوان ژورنال: Journal of Inherited Metabolic Disease
سال: 2010
ISSN: 0141-8955,1573-2665
DOI: 10.1007/s10545-010-9208-1