In vitro modelling of the neuropathophysiological features of mitochondrial epilepsy

Primary mitochondrial diseases are clinically and genetically heterogeneous inherited metabolic disorders. Such disorders are caused by pathogenic variants in mitochondrial DNA (mtDNA), the circular double-stranded 16,569 base pair DNA molecule that encodes 37 genes, and nuclear DNA genes that regulate mitochondrial function and structure [1,2]. Mitochondrial disorders may manifest at any age, affect any organ, and often have poor prognoses [3]. Neurological impairment, in particular epilepsy, is common in patients with primary mitochondrial disease [4].

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