Increased susceptibility to 4-HNE-induced toxicity and impaired development in a model of ALDH4A1-deficient pediatric epilepsy carrying the S352L variant.

Consistent with that observation, both S352L iNeurons and iAstrocytes had a 2-2.5-fold higher intracellular proline relative to isogenic iNeurons and iAstrocytes expressing WT ALDH4A1, though the increase in the proline levels of the iAstrocytes did not reach statistical significance (Fig. 3F ).

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