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The mutation in ALDH4A1 leads to the accumulation of pyrroline-5-carboxylate (P5C) in the mitochondria, which can react with PLP to cause PLP deficiency in the brain and can also be converted to proline, resulting in elevated plasma proline levels (Fig

The Keap1-BTB protein is an adaptor that bridges Nrf2 to a Cul3-based E3 ligase: oxidative stress sensing by a Cul3-Keap1 ligase