Acylcarnitine C5-OH Unit Converter

Acylcarnitine C5-OH (3-hydroxyisovalerylcarnitine) is a metabolic byproduct formed when the branched-chain amino acid leucine is broken down inside mitochondria. It is created when the intermediate molecule 3-hydroxyisovaleryl-CoA is conjugated with carnitine for transport out of the mitochondria.

This marker is most commonly measured as part of an acylcarnitine profile. Its levels reflect how efficiently your body processes leucine through the biotin-dependent enzyme 3-methylcrotonyl-CoA carboxylase (MCC).

C5-OH is included in newborn screening panels worldwide and is also used in adults to assess biotin status and screen for inherited metabolic disorders.

C5-OH is produced inside mitochondria throughout the body when leucine is broken down. It accumulates when the biotin-dependent enzyme 3-methylcrotonyl-CoA carboxylase (MCC) cannot efficiently process its substrate, and the resulting intermediate is conjugated with carnitine for export from the cell.