Pipecolic acid
- An insight into the biochemistry of inborn errors of metabolism for a clinical neurologist

Elevated levels of pipecolic acid, an intermediate in lysine metabolism, in the plasma and urine is characteristic of inherited defects of peroxisomal biogenesis. Pipecolic acid is usually determined by HPLC, gas chromatography or GC-MS. Plasma levels may also be elevated in children with hepatocellular disease or by ingestion of vegetables rich in pipecolate.

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