Carnitine palmitoyltransferase 1 (CPT1) controls the rate of entry of long-chain fatty acids into themitochondrial matrix for -oxidation and has been reported to exist as an oligomer. We have investigated the in vivo oligomerization of full-length rat CPT1A (rCPT1A) along with those of the N-terminal truncation/deletion mutants (1–82), (1–18), and (19–30) expressed in yeast mitochondria. The da...

A high prevalence of the sequence variant c.1436C→T in the CPT1A gene has been identified among Alaska Native newborns but the clinical implications of this variant are unknown. We conducted medically supervised fasts in 5 children homozygous for the c.1436C→T variant. Plasma free fatty acids increased normally in these children but their long-chain acylcarnitine and ketone production was signi...

Recent research has clarified many of the clinical applications of L-Carnitine and its related compounds, leading into new areas of potential use. Promising therapeutic applications of an ester form of carnitine, acetyl-L-Carnitine (ALC) are derived from observations that this compound readily crosses the bloodbrain barrier and improves neuronal energetics and repair mechanisms while modifying ...

Mersalyl inhibited the respiration of heart mitochondria under conditions that required the transport of (-)-carnitine and acyl(-)-carnitines. The exchange of external carnitine and acylcarnitines for intramitochondrial carnitine was also inhibited by mersalyl and 1 mM mersalyl proved suitable for the inhibitor-stop assay of carnitine acylcarnitine translocase. The carnitine-carnitine and (-)-c...

PURPOSE This study aimed to determine the expression and clinical significance of proteins that are involved in lipid metabolism in human breast tumors. METHODS Tumors from 476 breast cancer patients were used to construct tissue microarrays. Then, immunohistochemistry (IHC) for hormone-sensitive lipase (HSL), Perilipin 1 (PLIN1), fatty acid binding protein 4 (FABP4), carnitine palmitoyltrans...

Hypothalamic fatty acid metabolism is involved in central nervous system controls of feeding and energy balance. Malonyl-CoA, an intermediate of fatty acid biosynthesis, is emerging as a significant player in these processes. Notably, hypothalamic malonyl-CoA has been implicated in leptin's feeding effect. Leptin treatment increases malonyl-CoA level in the hypothalamic arcuate nucleus (Arc), a...