MICOS assembly controls mitochondrial inner membrane remodeling and crista junction redistribution to mediate cristae formation

ABSTRACT: Mitochondrial function is critically dependent on the folding of the mitochondrial inner membrane into cristae; indeed, numerous human diseases are associated to aberrant crista morphologies. With the MICOS complex, OPA1 and the F1Fo-ATP synthase, key players of crista biogenesis have been identified, yet their interplay is poorly understood. Harnessing super-resolution light and 3D electron microscopy, we dissect the roles of these proteins in the formation of cristae in human mitochondria. We individually disrupted the genes of all seven MICOS subunits in human cells and re-expressed Mic10 or Mic60 in the respective knockout cell line. We demonstrate that assembly of the MICOS complex triggers remodeling of pre-existing unstructured cristae and de-novo formation of crista junctions (CJs)

SUBMITTER: Prof. Stefan Jakobs 

PROVIDER: S-SCDT-EMBOJ-2019-104105 | biostudies-other |

REPOSITORIES: biostudies-other