How is L-carnitine synthesized?

Humans synthesize carnitine from the substrate TML (6-N-trimethyllysine), which is in turn derived from the methylation of the amino acid lysine. TML is then hydroxylated into hydroxytrimethyllysine (HTML) by trimethyllysine dioxygenase (TMLD), requiring the presence of ascorbic acid and iron.

Where is L-carnitine synthesized?

the liver
L-Carnitine is synthesized primarily in the liver but also in the kidneys and then transported to other tissues. It is most concentrated in tissues that use fatty acids as their primary fuel, such as skeletal and cardiac muscle.

What is the role of carnitine in fatty acid synthesis?

Carnitine is essential for the transfer of long-chain fatty acids across the inner mitochondrial membrane for subsequent β-oxidation. It can be synthesized by the body or assumed with the diet from meat and dairy products. Defects in carnitine biosynthesis do not routinely result in low plasma carnitine levels.

What is the proposed mechanism of action for L-carnitine?

Essentially, L-carnitine transports the chains of fatty acids into the mitochondrial matrix, thus allowing the cells to break down fat and get energy from the stored fat reserves. Recent studies have started to shed light on the beneficial effects of L-carnitine when used in various clinical therapies.

Which enzyme is involved in carnitine biosynthesis?

γ-Butyrobetaine hydroxylase is the key terminal rate-limiting enzyme in the pathway for biosynthesis of carnitine.

What is the purpose of carnitine?

Carnitine plays a critical role in energy production. It transports long-chain fatty acids into the mitochondria so they can be oxidized (“burned”) to produce energy. It also transports the toxic compounds generated out of this cellular organelle to prevent their accumulation.

What is the metabolic role of carnitine?

Carnitine has two principal functions in the organism. One is to transport long-chain fatty acids into the mitochondrion. The second function of carnitine is to regulate the intramitochondrial ratio of acylocoenzyme A to free coenzyme A.

How is L-carnitine absorbed?

Dietary L-carnitine is absorbed by active and passive transfer across enterocyte membranes. Bioavailability of dietary L-carnitine is 54-87% and is dependent on the amount of L-carnitine in the meal. Absorption of L-carnitine dietary supplements (0.5-6 g) is primarily passive; bioavailability is 14-18% of dose.

Whats L carnitine do?

Overview. L-carnitine is a chemical that is made in the human brain, liver, and kidneys. It helps the body turn fat into energy. L-carnitine is important for heart and brain function, muscle movement, and many other body processes.