3-Dehydrocarnitine

3-Dehydrocarnitine is a metabolite of the amino acid carnitine, which plays a crucial role in the metabolism of fatty acids. It is formed through the oxidation of carnitine by the enzyme carnitine dehydrogenase.

Structure and Properties

3-Dehydrocarnitine is a quaternary ammonium salt that contains three methyl groups, a carboxyl group, and a hydroxyl group. Its chemical formula is C7H15NO3 and its molecular weight is 161.20 g/mol.

Biological Role

In the body, 3-dehydrocarnitine is involved in the carnitine shuttle, a mechanism that transports fatty acids from the cytosol into the mitochondria for beta-oxidation. This process is essential for the production of ATP, the body's main source of energy.

Clinical Significance

Abnormal levels of 3-dehydrocarnitine in the body can be indicative of certain metabolic disorders, such as carnitine deficiency and carnitine uptake defect. These conditions can lead to symptoms such as muscle weakness, fatigue, and hypoglycemia.

See Also

• Carnitine
• Carnitine shuttle
• Carnitine deficiency
• Carnitine uptake defect