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3-OH-isovalerylcarnitine
3-OH-isovalerylcarnitine
Explanation: 3-OH-isovalerylcarnitine (3-hydroxyisovalerylcarnitine) is a compound naturally produced in the body during the breakdown of the amino acid leucine for energy. Normally, it’s present in small amounts, but high levels in blood can indicate problems with fatty acid oxidation or leucine processing, often linked to rare genetic disorders such as isovaleric acidemia or 3-methylcrotonyl-CoA carboxylase deficiency.
Test Purpose: Detect rare genetic disorders such as isovaleric acidemia or 3-methylcrotonyl-CoA carboxylase deficiency.
Other information about this test: Mayo Clinic, Cleveland Clinic, The University of Iowa, Department of Pathology
