MICOS and the mitochondrial inner membrane morphology – when things get out of shape

Mitochondria play a key role in cellular signalling, metabolism and energetics. Proper architecture remodelling of the inner mitochondrial membrane are essential for efficient respiration, apoptosis quality control cell. Several protein complexes including contact site cristae organizing system (MICOS), F1FO-ATP synthase, Optic Atrophy 1 (OPA1), facilitate formation, maintenance stability membranes. MICOS, OPA1 phospholipids such as cardiolipin phosphatidylethanolamine interact with each other to organize ultra-structure remodel response cell's demands. Functional alterations these proteins or biosynthesis pathway result an aberrant impair function. Mitochondrial dysfunction abnormalities hallmark several human conditions diseases neurodegeneration, cardiomyopathies diabetes mellitus. Yet, they have long been regarded secondary pathological effects. This review discusses emerging evidence direct relationship between protein- lipid-dependent regulation morphology fatal encephalopathy, Leigh syndrome, Parkinson's disease, cancer.