Pipecolic acid

Abstract

Pipecolic acid (piperidine-2-carboxylic acid), an imino acid, is an intermediate in the lysine degradation pathway. Its analysis in urine, plasma, and CSF has two diagnostic points of relevance, i.e. giving additional evidence of AASA dehydrogenase deficiency in pyridoxine responsive convulsions, and enabling the distinction between generalized peroxisome biogenesis defects and defects of individual peroxisomal enzymes. Pipecolic acid can be analyzed using tandem mass spectrometry and stable isotope dilution gas chromatography / mass spectrometry. The amino acid analyzer approach has insufficient sensitivity. © 2008 Springer-Verlag.