Respiratory supercomplexes act as a platform for complex III-mediated maturation of human mitochondrial complexes I and IV

ABSTRACT: Mitochondrial respiratory chain (MRC) enzymes associate in supercomplexes (SCs) that are structurally interdependent. This may explain why defects in a single component often produce combined enzyme deficiencies in patients. A case in point is the alleged destabilization of complex I in the absence of complex III. To clarify the structural and functional relationships between complexes, we have used comprehensive proteomic, functional and biogenetical approaches to analyse a MT-CYB-deficient human cell line. We show that absence of complex III blocks complex I biogenesis by preventing the incorporation of the NADH-module rather than decreasing its stability. In addition, complex IV subunits appeared sequestered within complex III subassemblies, leading to defective complex IV assembly as w

SUBMITTER: Ms. Margherita Protasoni 

PROVIDER: S-SCDT-EMBOJ-2019-102817 | biostudies-other |

REPOSITORIES: biostudies-other