A diverse range of uncommon genetic abnormalities known as mitochondrial diseases can result from mutations in either the nuclear (nDNA) or mitochondrial DNA (mtDNA). Multiple maternally inherited genetic illnesses are linked to mtDNA mutations, with mitochondrial dysfunction as a key clinical trait. Although typically multisystemic, these disorders mostly affect the brain and skeletal muscles, two organs that demand a lot of energy. The discovery of induced pluripotent stem cells (iPSCs), in contrast to the challenge of acquiring neuronal and muscle cell models, has aided in the understanding of mitochondrial illnesses. A suitable cellular model is still difficult to come by, which makes it difficult to develop novel treatments for those who suffer from these disorders. In this review, we expand our understanding of the current therapeutic models for the two most well-studied mt-tRNA mutation-caused mitochondrial diseases, MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) and MERRF (myoclonic epilepsy with ragged red fibres) syndromes. We will focus on the creation of induced neurons (iNs), which are produced by direct reprogramming of fibroblasts taken from individuals with the MERRF syndrome. This innovative paradigm for studying mitochondrial diseases will be discussed in particular. Because iNs might replicate the pathophysiology of patients' neurons and provide us with the chance to fix the flaws, we believe that they will be useful for modelling mitochondrial illness. Since iNs could imitate patients' neuron pathophysiology and provide us with the chance to fix the changes in one of the most impacted cellular types in these disorders, we anticipate that iNs will be useful for modelling mitochondrial disease.
