The mainstay of initial treatment is glucose infusion at a rate greater than 7 mg/kg/minute, which inhibits beta-oxidation of fatty acids (the defective enzymatic steps in carnitine-acylcarnitine translocase deficiency) and thus prevents the accumulation of toxic long-chain acylcarnitines. 展开 ...
2.2SCFAs – revisiting the carnitine shuttle Carnitinehas also been proposed to have an important buffering role to regulate the amount of CoA that is acyl-bound or free to engage additional substrates (e.g. Ref.[51]), and also has antioxidant properties by virtue of its ability to scavenge...
The oxidation of medium- (C6–C10) and short-chain (C4–C6) fatty acids seems largely independent from the carnitine shuttle [19], [66]. Under physiologic conditions, oxidation of long- and medium-chain fatty acids is primarily handled by the mitochondrial β-oxidation system, with only ...
re-imported into the mitochondria for beta-oxidation (Futile Cycle). ALCAR, shuttled to the nucleus (shown in violet), can be used as source of acetyl groups for histone acetylation, probably contributing to lipid metabolism-specific gene expression Small-non-coding RNA (miRNAs) as a carnitine ...
ALCAR, shuttled to the nucleus (shown in violet), can be used as source of acetyl groups for histone acetylation, probably contributing to lipid metabolism-specific gene expression Full size image Small-non-coding RNA (miRNAs) as a carnitine system regulator Among all small non-coding RNAs, ...
chain fatty acids, activated to their CoA esters in the cytosol, are shuttled across the barrier of the inner mitochondrial membrane by the carnitine ... M.,Brivet,A.,... - 《Journal of Inherited Metabolic Disease》 被引量: 210发表: 1999年 Carnitine-acylcarnitine translocase deficiency is ...
(OS) at systemic, intraovarian, and intrafollicular levels is one of the main factors involved in the pathogenesis of PCOS. We investigated the ability of different acyl-carnitines to act at the oocyte level by counteracting the effects of OS on carnitine shuttle system and mitochondrial ...