Acylcarnitine C14:1 Unit Converter

Acylcarnitine C14:1 (tetradecenoylcarnitine) is a long-chain acylcarnitine formed when a 14-carbon unsaturated fatty acid binds to carnitine. It serves as a transport molecule that shuttles long-chain fatty acids into the mitochondria, where they are broken down for energy through a process called beta-oxidation.

C14:1 is primarily used as a screening marker for very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency, a rare inherited disorder of fatty acid metabolism. When VLCAD enzyme activity is reduced, the body cannot efficiently break down long-chain fats, causing C14:1 to accumulate in the blood.

It is typically measured as part of a broader acylcarnitine profile and interpreted alongside related markers such as C14, C14:2, C12:1, and ratios like C14:1/C2 and C14:1/C16.

C14:1 is produced in cells throughout the body when long-chain unsaturated fatty acids combine with carnitine via the enzyme carnitine palmitoyltransferase I (CPT-I) on the outer mitochondrial membrane. The liver, skeletal muscle, and heart are the primary sites of long-chain fatty acid oxidation.