karnitiini

Karnitiini is a four-carbon organic compound that plays a crucial role in the metabolism of fatty acids. It is a derivative of lysine, an essential amino acid, and is synthesized in the body through the action of the enzyme lysine-ketoglutarate reductase. Karnitiini is primarily found in the mitochondria, where it acts as a shuttle to transport long-chain fatty acids into the mitochondrial matrix for beta-oxidation, a process that breaks down fatty acids to generate energy.

The primary function of karnitiini is to facilitate the transport of long-chain fatty acids across the mitochondrial inner membrane. This process involves the formation of acyl-karnitiini esters, which are then transported into the mitochondrial matrix. Inside the mitochondria, the fatty acids are broken down into acetyl-CoA, which enters the citric acid cycle to produce energy in the form of ATP.

Karnitiini deficiency can lead to various metabolic disorders, including primary carnitine deficiency, which is characterized by symptoms such as muscle weakness, fatigue, and developmental delays. Treatment typically involves dietary supplementation with karnitiini or its precursors, such as lysine.

Karnitiini is also used as a dietary supplement to support energy production and muscle function. It is commonly found in various nutritional products, including energy bars, sports drinks, and dietary supplements. However, the effectiveness of karnitiini supplements for general health and athletic performance is a topic of ongoing research and debate.