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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-Oxoisovaleric acid forms from valine breakdown, one of the branched-chain amino acids, with processing relying on B-vitamin-powered enzymes including thiamine (B1).
