Alpha Keto Isoleucine
3-Methyl-2-oxovaleric acid is a metabolite of Alpha Keto Isoleucine, Alpha Keto Isoleucine Calcium Saltupon it is further degraded by branched chain keto acid
dehydrogenase E1 to .
It is used as a clinical marker for maple syrup urine disease (MSUD).
MSUD is caused by a deficiency of the branched-chain -keto acid
dehydrogenase compex resulting in an accumulation of branched-chain
amino acids and the corresponding -keto-and -hydroxy acids in blood,
urine and cerebrospinal fluid causing neurological damage and mental