The mitochondrial respiratory chain consists of 5 enzyme complexes that are responsible for ATP generation.The paradigm of the electron transport chain as discrete enzymes diffused in the inner mitochondrial membrane ...The mitochondrial respiratory chain consists of 5 enzyme complexes that are responsible for ATP generation.The paradigm of the electron transport chain as discrete enzymes diffused in the inner mitochondrial membrane has been replaced by the solid state supercomplex model wherein the respiratory complexes associate with each other to form supramolecular complexes.Defects in these supercomplexes,which have been shown to be functionally active and required for forming stable respiratory complexes,have been associated with many genetic and neurodegenerative disorders demonstrating their biomedical signifi cance.In this review,we will summarize the functional and structural signifi cance of supercomplexes and provide a comprehensive review of their assembly and the assembly factors currently known to play a role in this process.展开更多
基金The relevant work carried out in the authors’laboratory has been supported by grants from National Institute of Health(R21 NS072777)the Morrison Trust.
文摘The mitochondrial respiratory chain consists of 5 enzyme complexes that are responsible for ATP generation.The paradigm of the electron transport chain as discrete enzymes diffused in the inner mitochondrial membrane has been replaced by the solid state supercomplex model wherein the respiratory complexes associate with each other to form supramolecular complexes.Defects in these supercomplexes,which have been shown to be functionally active and required for forming stable respiratory complexes,have been associated with many genetic and neurodegenerative disorders demonstrating their biomedical signifi cance.In this review,we will summarize the functional and structural signifi cance of supercomplexes and provide a comprehensive review of their assembly and the assembly factors currently known to play a role in this process.
