Structural rather than catalytic role for mitochondrial respiratory chain supercomplexes

Author:

Brischigliaro Michele12ORCID,Cabrera-Orefice Alfredo3ORCID,Arnold Susanne34,Viscomi Carlo12,Zeviani Massimo5,Fernández-Vizarra Erika12ORCID

Affiliation:

1. Department of Biomedical Sciences, University of Padova

2. Veneto Institute of Molecular Medicine

3. Radboud Institute for Molecular Life Sciences, Radboud University Medical Center

4. Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD), University of Cologne

5. Department of Neurosciences, University of Padova

Abstract

Mammalian mitochondrial respiratory chain (MRC) complexes are able to associate into quaternary structures named supercomplexes (SCs), which normally coexist with non-bound individual complexes. The functional significance of SCs has not been fully clarified and the debate has been centered on whether or not they confer catalytic advantages compared with the non-bound individual complexes. Mitochondrial respiratory chain organization does not seem to be conserved in all organisms. In fact, and differently from mammalian species, mitochondria from Drosophila melanogaster tissues are characterized by low amounts of SCs, despite the high metabolic demands and MRC activity shown by these mitochondria. Here, we show that attenuating the biogenesis of individual respiratory chain complexes was accompanied by increased formation of stable SCs, which are missing in Drosophila melanogaster in physiological conditions. This phenomenon was not accompanied by an increase in mitochondrial respiratory activity. Therefore, we conclude that SC formation is necessary to stabilize the complexes in suboptimal biogenesis conditions, but not for the enhancement of respiratory chain catalysis.

Funder

Fondazione Telethon

French Muscular Dystrophy Association

Università degli Studi di Padova

Publisher

eLife Sciences Publications, Ltd

Subject

General Immunology and Microbiology,General Biochemistry, Genetics and Molecular Biology,General Medicine,General Neuroscience

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