L-Pipecolic acid is a proline homolog and lysine metabolite. It occurs in seeds, malt, edible mushrooms, fruits, etc. Defects in its metabolism is involved in hyperpipecolic acidemia, cerebro-hepato-renal syndrome, neonatal onset adrenoleukodystrophy, and infantile Refsum disease. L-pipecolic acid (L-PA) is use as an important biochemical marker for the diagnosis of pero x isomal disorders. L-Pipecolic acid is a lysine metabolite; defect in its catabolism is involved in hyperpipecolic acidemia,...