Mitochondrial Bol1 and Bol3 function as assembly factors for specific iron-sulfur proteins

Author:

Uzarska Marta A1,Nasta Veronica2,Weiler Benjamin D1,Spantgar Farah1,Ciofi-Baffoni Simone23,Saviello Maria Rosaria23,Gonnelli Leonardo23,Mühlenhoff Ulrich1,Banci Lucia23,Lill Roland14ORCID

Affiliation:

1. Institut für Zytobiologie und Zytopathologie, Philipps-Universität, Marburg, Germany

2. Magnetic Resonance Center CERM, University of Florence, Florence, Italy

3. Department of Chemistry, University of Florence, Florence, Italy

4. LOEWE Zentrum für Synthetische Mikrobiologie SynMikro, Marburg, Germany

Abstract

Assembly of mitochondrial iron-sulfur (Fe/S) proteins is a key process of cells, and defects cause many rare diseases. In the first phase of this pathway, ten Fe/S cluster (ISC) assembly components synthesize and insert [2Fe-2S] clusters. The second phase is dedicated to the assembly of [4Fe-4S] proteins, yet this part is poorly understood. Here, we characterize the BOLA family proteins Bol1 and Bol3 as specific mitochondrial ISC assembly factors that facilitate [4Fe-4S] cluster insertion into a subset of mitochondrial proteins such as lipoate synthase and succinate dehydrogenase. Bol1-Bol3 perform largely overlapping functions, yet cannot replace the ISC protein Nfu1 that also participates in this phase of Fe/S protein biogenesis. Bol1 and Bol3 form dimeric complexes with both monothiol glutaredoxin Grx5 and Nfu1. Complex formation differentially influences the stability of the Grx5-Bol-shared Fe/S clusters. Our findings provide the biochemical basis for explaining the pathological phenotypes of patients with mutations in BOLA3.

Funder

Deutsche Forschungsgemeinschaft

European Commission

European Strategy Forum on Research Infrastructures

LOEWE program of state Hesse, Germany

Von-Behring-Röntgen-Stiftung

Publisher

eLife Sciences Publications, Ltd

Subject

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

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