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Our approach is a simple and promising strategy to evaluate the effects of heat stress or therapeutic drugs on mitochondrial FAO. 展开 关键词: Heat stress Bezafibrate Mitochondrial fatty acid β-oxidation disorder In vitro Acylcarnitine profiling ...
Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. Pediatr. Res. 56, 744-750.Tyni, T., Paetau, A., Strauss, A. W., Middleton, B., and Kivela, T. (2004) Mitochondrial fatty acid...
While the pathways of diatom fatty acid and TAG synthesis appear to be well conserved with plants, the pathways of TAG catabolism and downstream fatty acid beta-oxidation have not been characterised in diatoms.Jallet, DenisXing, DenghuiHughes, AlexanderMoosburner, MarkSimmons, Mark P.Allen, ...
A new genetic disorder in mitochondrial fatty acid beta-oxidation: ACAD9 deficiency. Am J Hum Genet. 2007; 81(1):87-103. [PubMed: 17564966]He M , Rutledge SL , Kelly DR , Palmer CA , Murdoch G , Majumder N , et al . 2007 . A new genetic disorder in mitochondrial fatty acid ...
Pigmentary retinopathy is an important feature of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, a disorder of mitochondrial fatty acid beta-oxidation. Pathogenesis of this complication remains unknown. The retinal pigment epithelium (RPE) is affected early in this retinopathy. We wanted...
The methods modulate the mitochondrial fatty acid β-oxidation pathway at the last step so that the product of the mutant protein accumulates and stabilizes the mutant protein and/or the substrate(s)/product(s) of the down stream reactions accumulate and possibly bind to allosteric sites on the...
The methods modulate the mitochondrial fatty acid β-oxidation pathway at the last step so that the product of the mutant protein accumulates and stabilizes the mutant protein and/or the substrate(s)/product(s) of the down stream reactions accumulate and possibly bind to allosteric sites on the...
Bastin, JBastin J. Regulation of mitochondrial fatty acid ß-oxidation in human: what can we learn from inborn fatty acid ß-oxidation deficiencies? Biochimie (2014) 96:113-20. doi:10.1016/j.biochi.2013.05.012