Mitochondrial protein import dysfunction: mitochondrial disease, neurodegenerative disease and cancer

The majority of proteins localised to mitochondria are encoded by the nuclear genome, with approximately 1500 imported into mammalian mitochondria. Dysfunction in this fundamental cellular process is linked a variety pathologies including neuropathies, cardiovascular disorders, myopathies, neurodegenerative diseases and cancer, demonstrating importance mitochondrial protein import machinery for function. Correct requires co-ordinated activity multimeric translocation sorting machineries located both outer inner membranes, directing destined compartment. This dynamic maintains homeostasis, its dysregulation significantly affects signalling pathways metabolism. review summarises current knowledge pathological consequences mutation components. In addition, we will discuss role our understanding Alzheimer's disease, Huntington's disease Parkinson's disease.