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Biology Articles » Biochemistry » An insight into the biochemistry of inborn errors of metabolism for a clinical neurologist » Organic acids

Organic acids
- An insight into the biochemistry of inborn errors of metabolism for a clinical neurologist

Organic acids comprise key metabolites of virtually all pathways of intermediary metabolism as well as exogenous compounds. They are generally determined in urine by the gas chromatography-mass spectrometry (GC-MS), although diagnostically important organic acid changes occur in a variety of physiological fluids. Preparation of the analysis includes preliminary extraction of the organic acids from urine into an organic solvent; this is followed by derivatization to form trimethysilyl derivatives to lower the temperature at which they vaporise and to prevent them from undergoing thermal decomposition, before injecting into the GC-MS. The preliminary identification of the organic acids is based on the retention time, that is, the time taken by the compound to pass through the column. The interpretation of organic acid analysis depends on several key diagnostic metabolites as well as the characteristic patterns of abnormalities.

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