Mitochondrial fatty acid 尾 -oxidation is the primary pathway for metabolizing fatty acids with chain lengths ranging from 4 to 20 carbons. This pathway is necessary for energy production during times when glucose may be low or unavailable, particularly for those tissues relying predominately on ...
The results give strong in vitro evidence for the presence of mitochondrial fatty acid beta-oxidation in RPE cells and the expression of the MTP in the RPE and other layers of the retina. Further studies are required to clarify whether this pathway acts also in vivo in the retina. 展开 ...
Malonyl-CoA is a cellular substrate that can inhibit fatty acid β-oxidation in the mitochondria through allosteric inhibition of carnitine palmitoyltransferase 1A (CPT1A), the enzyme that controls the rate-limiting step of the long chain fatty acid β-oxidation cycle. We hypothesized that changes ...
Inherited defects in mitochondrial fatty-acid beta-oxidation comprise a group of at least 12 diseases characterized by distinct enzyme or transporter deficiencies. Most of these diseases have a variable age of onset and clinical severity. Symptoms are often episodic and associated with mild viral illne...
Mitochondrial β-oxidation of fatty acids generates acetyl-coA, NADH and FADH 2 . Acyl-coA synthetases catalyze the binding of fatty acids to coenzyme A to form fatty acyl-coA thioesters, the first step in the intracellular metabolism of fatty acids. l -carnitine system facilitates the transport...
J. The biochemistry and physiology of mitochondrial fatty acid beta-oxidation and Its genetic disorders. Annu. Rev. Physiol. 78, 23–44 (2016). Article CAS PubMed Google Scholar Bennett, M. J., Sheng, F. & Saada, A. Biochemical assays of TCA cycle and beta-oxidation metabolites. ...
Ouali F, Djouadi F, Merlet-Benichou C, Riveau B, Bastin J (2000) Regulation of fatty acid transport protein and mitochondrial and peroxisomal beta-oxidation gene expression by fatty acids in developing rats. Pediatr Res 48:691...
Here, we found that a high-fat diet (HFD) enhanced mitochondrial fatty acid β-oxidation, which enhanced acetyl-CoA levels in the liver of the large yellow croaker. The HFD activated ACLY to govern the "citrate transport" to transfer acetyl-CoA from the mitochondria to the nucleus. Elevated...
Fatty acid oxidation (FAO)-driven H2O2 has been shown to be a major source of oxidative stress in several tissues and disease states. Here, we established that the mitochondrial flavoprotein long-chain acyl-CoA dehydrogenase (LCAD), which catalyzes a key step in mitochondrial FAO, directly produ...
Moreover, HFD mice displayed a downregulated expression of genes involved in lipolysis and fatty acid oxidation in the liver (Supplementary Fig. S1L). Hepatic HtrA2/Omi expression, the rate-limiting enzyme of β-oxidation, and autophagy-associated proteins were decreased during NAFLD progression Htr...