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Fumaric acid accumulation also inhibits the activity of adenylosuccinate lyase (ADSL) in the purine de novo synthesis pathway, resulting in an increase in succinylaminoimidazolecarboxamide ribose (SAICAR) and adenosine succinate, which can cause damage to the central nervous system [84]

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Mitochondrial dysfunction in CA1 hippocampal neurons of the UBE3A deficient mouse model for Angelman syndrome