L-Pipecolic Acid

L-Pipecolic acid is a metabolite of the amino acid L-lysine.¹ L-Pipecolic acid is oxidized to L-α-aminoadipic acid by α-aminoadipic semialdehyde dehydrogenase (α-AASA dehydrogenase) and peroxisomal sarcosine oxidase (PSO).^2,3 L-Pipecolic acid inhibits GABA binding in bovine brain homogenate in the presence, but not absence, of hexobarbital or pentobarbital (IC₅₀s = 0.2 and 2 nM, respectively).⁴ Serum levels of L-pipecolic acid are elevated in patients with Zellwager syndrome, a peroxisomal condition characterized by hepatomegaly, abnormal facial features, and cognitive impairments.⁵

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1. Chang, Y.F. Pipecolic acid pathway: The major lysine metabolic route in the rat brain. Biochem. Biophys. Res. Commun. 69(1), 174-180 (1976).

2. Chang, Y.F., Ghosh, P., and Rao, V.V. L-pipecolic acid metabolism in human liver: L-α-aminoadipate δ-semialdehyde oxidoreductase. Biochim. Biophys. Acta 1038(3), 300-305 (1990).

3. Dodt, G., Kim, D.G., Reimann, S.A., et al. L-Pipecolic acid oxidase, a human enzyme essential for the degradation of L-pipecolic acid, is most similar to the monomeric sarcosine oxidases. Biochem. J. 345 Pt. 3(Pt. 3), 487-494 (2000).

4. Feigenbaum, P., and Chang, Y.F. Pipecolic acid antagonizes barbiturate-enhanced GABA binding to bovine brain membranes. Brain Res. 372(1), 176-179 (1986).

5. Mihalik, S.J., Moser, H.W., Watkins, P.A., et al. Peroxisomal L-pipecolic acid oxidation is deficient in liver from Zellweger syndrome patients. Pediatr. Res. 25(5), 548-552 (1989).