Most fatty acids in the human body are long-chain fatty acids. The β-oxidation of fatty acids occurs in the mitochondria. After activation of long-chain fatty acids, they need assistance from carnitine as a carrier and catalysis of carnitine palmitoyltransferase I (CPT I) in the outer mitochondrial membrane and carnitine palmitoyltransferase II (CPT II) in the inner mitochondrial membrane to en...

Running title: He et al.; CPT1b deficiency aggravates pressure-overload-induced cardiac hypertrophy Abstract: Background-Carnitine palmitoyltransferase 1(CPT1) is a rate-limiting step of mitochondrial E

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...

Mitochondrial fatty acid beta-oxidation is important for energy production, which is stressed by the different defects found in this pathway. Most of the enzyme deficiencies causing these defects are well characterized at both the protein and genomic levels. One exception is carnitine palmitoyltransferase I (CPT I) deficiency, of which until now no mutations have been reported although the defe...

Salicylyl-CoA and benzoyl-CoA were good inhibitors of carnitine acetyltransferase (CAT), competing with acetyl-CoA with Ki values of 7.5 and 22 microM respectively in the forward direction and with CoA in the reverse reaction with similar Ki values. They were also competitive inhibitors of carnitine octanoyltransferase (Ki = 261 and 295 microM respectively), but were only weakly inhibitory to c...

The purpose of this study was to characterize the mitochondrial carnitine octanoyltransferase of beef heart. Carnitine acyltransferase activities were solubilized from isolated beef heart mitochondria using KC1 and the nonionic detergent, Triton X-100, at final concentrations of 1 M and 2%, respectively. Upon fractionation of the solubilized protein on Cibacron Blue Sepharose, two protein peaks...

Background. This study was designed to evaluate the effect of severe peripheral arterial insufficiency on carnitine concentrations and carnitine acetyltransferase and palmitoyltransferase activities in the ischemic skeletal muscles of patients with severe peripheral vascular disease. Methds and Results. Nine biopsy specimens ofischemic muscles were obtained from five patients undergoing reconst...