Condensation of delta-1-piperideine-6-carboxylate with ortho-aminobenzaldehyde allows its simple, fast, and inexpensive quantification in the urine of patients with antiquitin deficiency.

All patients tested in this study were treated with pyridoxine and some additionally with a lysine‐restricted diet, but the difference between determined control and patient urine P6C concentrations was statistically highly significant.

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