Acylcarnitine C18:1-OH Unit Converter

Acylcarnitine C18:1-OH, also called 3-hydroxyoleoylcarnitine, is a long-chain hydroxylated acylcarnitine. It is formed when 3-hydroxyoleoyl-CoA, an intermediate of long-chain fatty acid beta-oxidation, is conjugated with L-carnitine.

This marker reflects the activity of the final steps of mitochondrial long-chain fatty acid oxidation. When the enzymes responsible for processing long-chain 3-hydroxy fatty acid intermediates are impaired, C18:1-OH accumulates in the blood.

It is primarily used as a screening and diagnostic marker for inherited disorders of fatty acid oxidation, particularly long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency and mitochondrial trifunctional protein (TFP) deficiency.

C18:1-OH is produced in the mitochondria of cells throughout the body as an intermediate of long-chain fatty acid beta-oxidation. It accumulates when the enzyme LCHAD or the mitochondrial trifunctional protein complex cannot process it further.