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Fig. 1 Pipecolic acid (left) and saccharopine (right) pathways for L-lysine catabolism in animals. The L-pipecolic acid pathway. LKR (lysine-ketoglutarate reductase) domain and SDH (saccharopine dehydrogenase) domain of the bifunctional AASS (aminoadipic semialdehyde synthase) enzyme. Antiquitin (encoded by the gene ALDH7A1) is deficient in pyridoxine-dependent epilepsy (red) leading to accumulation of AASA and P6C (red arrows). PIPOX (pipecolic acid oxidase). P5CR (piperideine-5-carboxilic reductase), encoded by the gene PYCR1. Further enzymatic steps convert AAA to Acetyl-CoA.
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