The effect of omeprazole on the mitochondrial carnitine/acylcarnitine transporter has been studied in proteoliposomes. Externally added omeprazole inhibited the carnitine/carnitine antiport catalysed by the transporter. The inhibition was partially reversed by DTE indicating that it was caused by the covalent reaction of omeprazole with Cys residue(s). Inhibition of the C-less mutant transporte...

Carnitine is a conditionally essential nutrient that plays a vital role in energy production and fatty acid metabolism. Vegetarians possess a greater bioavailability than meat eaters. Distinct deficiencies arise either from genetic mutation of carnitine transporters or in association with other disorders such as liver or kidney disease. Carnitine deficiency occurs in aberrations of carnitine re...

have been reported by several authors [2,3,6 ]. The Introduction high total serum concentration of carnitine results predominantly from an increase in -carnitine esters, Recombinant human erythropoietin (rhEpo) is widely i.e. acylcarnitine. As a result of the reduction in free used for correction of anaemia in patients with endcarnitine, the ratios of free/total carnitine and free stage renal ...

INTRODUCTION L-carnitine is an important metabolic mediator involved in fatty acid transport. It is obtained from the diet, particularly from animal products, such as red meat. Previous reports have revealed that long-term tube feeding with a commercial product containing no or low levels of carnitine can lead to an altered mental state caused by hyperammonemia. CASE PRESENTATION A 72-year-ol...

Levo-carnitine (L-carnitine) facilitates the transport of long chain fatty acyl co-enzyme A (CoA) across the mitochondrial membrane for eventual oxidation and energy production. Carnitine deficiency results in free fatty acid accumulation and contributes to cardiovascular complications in experimental diabetes. In this condition, oral carnitine supplementation provides cardio-protection by its ...

A 3 year old boy developed an unusually mild form of glycogen storage disease type IV. Metabolic investigations showed severe abnormalities of fatty acid and carnitine metabolism. A muscle carnitine deficiency was found. Treatment with L-carnitine orally led to a notable improvement in muscle strength.

L‐carnitine (free carnitine) transports cytosolic long‐chain fatty acids as acylcarnitines across the inner mitochondrial membrane for β‐oxidation and subsequent ATP production in the mitochondria. The endogenous carnitine pool comprises free L-carnitine as well as short-, mediumand long-chain fatty acid esters (acyl-carnitines) and depends on absorption of L-carnitine from dietary sources, end...

Despite an abundance of literature describing the basic mechanisms of action of L-carnitine metabolism, there remains some uncertainty regarding the effects of oral L-carnitine supplementation on in vivo fatty acid oxidation in normal subjects under normal conditions. It is well known that L-carnitine normalizes the metabolism of long-chain fatty acids in cases of carnitine deficiency. However,...

BACKGROUND AND AIMS Low free testosterone levels are associated with sexual dysfunction and an increased risk of cardiovascular disease in male hemodialysis patients. Carnitine deficiency is frequently observed in hemodialysis patients as well. However, the relationship between carnitine and testosterone levels remains unknown. In this study, we examined whether carnitine deficiency was indepen...

The authors investigated 32 patients with the muscle form of CPT II deficiency. Total carnitine palmitoyltransferase enzyme system (CPT) activity was normal but abnormally inhibited by malonyl-CoA, palmitoyl-CoA, and the detergents Triton X and Tween 20. Mutation analysis identified three described mutations (S113L, P50H, and F448L) and two novel mutations (M214T and Y479F). Using modeling tech...