Synonyms: (2R)-3-Carboxy-2-(3-hydroxy-3-methyl-1-oxobutoxy)-N,N,N-trimethyl-1-propanaminium inner salt
Molecular Formula: C12H23NO5
Molecular Weight: 261.31
Linear Structural Formula: C12H23NO5
Purity: >=90.0% (HPLC)
Storage: 2-8C
Biochem Physiol Actions: 3-Hydroxyisovaleryl-L-carnitine is an endogenous metabolite that can result from impairment in the leucine catabolic pathway. 3-Hydroxyisovaleryl-L-carnitine was observed in plasma and urine of patient with isolated 3-methylcrotonyl-CoA carboxylase deficiency and is used as biomarker of biotin deficiency in humans and other metabolic disorders.