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. Author manuscript; available in PMC: 2018 Nov 1.
Published in final edited form as: Pediatr Neurol. 2017 Jun 3;76:47–53. doi: 10.1016/j.pediatrneurol.2017.05.024

FIGURE 1.

FIGURE 1

B6 metabolism. Pyridoxine and pyridoxamine are derived from vegetables and meat, respectively. These two are ultimately converted into pyridoxal-5-phosphate (P5P) by PNPO. Defects in antiquitin, an enzyme in the lysine degradation pathway, are the cause of pyridoxine-responsive seizures leading to accumulation of the upstream intermediary alpha-aminoadipic semialdehyde (AASA) and sequestration of P5P. P6C, piperideine-6-carboxylic acid; ALDH7A1, antiquitin or aldehyde dehydrogenase 7 family, member A1.