Fine-tuning of the respiratory complexes stability and supercomplexes assembly in cells defective of complex III.

ABSTRACT: The respiratory complexes are organized in supramolecular assemblies called supercomplexes thought to optimize cellular metabolism under physiological and pathological conditions. In this study, we used genetically and biochemically well characterized cells bearing the pathogenic microdeletion m.15,649-15,666 (ΔI300-P305) in MT-CYB gene, to investigate the effects of an assembly-hampered CIII on the re-organization of supercomplexes. First, we found that this mutation also affects the stability of both CI and CIV, and evidences the occurrence of a preferential structural interaction between CI and CIII₂, yielding a small amount of active CI+CIII₂ supercomplex. Indeed, a residual CI+CIII combined redox activity, and a low but detectable ATP synthesis driven by CI subst

SUBMITTER: Tropeano CV 

PROVIDER: S-EPMC8439561 | biostudies-literature | 2020 Feb

REPOSITORIES: biostudies-literature