A novel method for simultaneous quantification of alpha-aminoadipic semialdehyde/piperideine-6-carboxylate and pipecolic acid in plasma and urine
T Yuzyuk, A Liu, A Thomas, JDE Wilson… - … of Chromatography B, 2016 - Elsevier
Objectives Elevated levels of pipecolic acid (PA), α-aminoadipic semialdehyde (AASA) and
its cyclic form Δ1-piperideine-6-carboxylate (P6C) are characteristic of pyridoxine dependent
epilepsy (PDE), a rare disorder of inborn error of metabolism. Recent studies showed the
effectiveness of dietary therapy in PDE patients and emphasized the importance of the
assessment of these metabolites for monitoring treatment efficacy. The objective of this study
was to develop a robust and sensitive method for simultaneous quantification of AASA-P6C …
its cyclic form Δ1-piperideine-6-carboxylate (P6C) are characteristic of pyridoxine dependent
epilepsy (PDE), a rare disorder of inborn error of metabolism. Recent studies showed the
effectiveness of dietary therapy in PDE patients and emphasized the importance of the
assessment of these metabolites for monitoring treatment efficacy. The objective of this study
was to develop a robust and sensitive method for simultaneous quantification of AASA-P6C …