Method: GC-MS (Gas Chromatography/Mass Spectrometry) measuring organic acid metabolites in urine (CLIA 17D0919496); not cleared or approved by the FDA. Results in mmol/mol creatinine. Not a stand-alone diagnosis; should be interpreted in clinical context.
2-Hydroxyisocaproic acid comes from processing leucine, one of the branched-chain amino acids, with breakdown leaning on B-vitamin-dependent enzymes including thiamine.



