Fig. 2
From: Precision diagnosis and treatment of vitamin metabolism-related epilepsy

Lysine metabolism. ALDH7A1 is involved in the oxidation of lysine and mutations in this gene lead to pathological accumulation of α-AASA, P6C and piperonylic acid. P6C accumulation can react with PLP to form a Knoevenagel condensate, which inactivates PLP and interferes with the metabolism of important neurotransmitters in the brain