Mitochondrial dysfunction in cognitive neurodevelopmental disorders: Cause or effect?

•Mitochondria-mediated energy homeostasis is crucial for brain functions and cognition.•Aberrant mitophagy, respiratory chain deficits, and oxidative stress resulting from mitochondrial dysfunction could lead to aberrant neurodevelopment.•Detailed studies are required to effectively answer the long-standingof whether mitochondrial dysfunction is a cause or a consequence of neurodevelopmental disorders.•Mitochondria are considered as potential therapeutic targets for neurodevelopmental disorders. Mitochondria have a crucial role in brain development and neurogenesis, both in embryonic and adult brains. Since the brain is the highest energy consuming organ, it is highly vulnerable to mitochondrial dysfunction. This has been implicated in a range of brain disorders including, neurodevelopmental conditions, psychiatric illnesses, and neurodegenerative diseases. Genetic variations in mitochondrial DNA (mtDNA), and nuclear DNA encoding mitochondrial proteins, have been associated with several cognitive disorders. However, it is not yet clear whether mitochondrial dysfunction is a primary cause of these conditions or a secondary effect. Our review article deals with this topic, and brings out recent advances in mitochondria-oriented therapies. Mitochondrial dysfunction could be involved in the pathogenesis of a subset of disorders involving cognitive impairment. In these patients, mitochondrial dysfunction could be the cause of the condition, rather than the consequence. There are vast areas in this topic that remains to be explored and elucidated.