2NCL

Solution structure of BOLA3 from Homo sapiens

Summary for 2NCL

• Entry DOI: 10.2210/pdb2ncl/pdb
• NMR Information: BMRB: 26031
• Descriptor: BolA-like protein 3 (1 entity in total)
• Functional Keywords: class ii kh-like fold, mitochondrial protein, fe/s protein biogenesis, protein binding
• Biological source: Homo sapiens (human)
• Cellular location: Secreted : Q53S33
• Total number of polymer chains: 1
• Total formula weight: 9306.76

Authors

Ciofi-Baffoni, S.,Nasta, V.,Banci, L. (deposition date: 2016-04-11, release date: 2016-09-07, Last modification date: 2024-05-15)

Primary citation

Uzarska, M.A.,Nasta, V.,Weiler, B.D.,Spantgar, F.,Ciofi-Baffoni, S.,Saviello, M.R.,Gonnelli, L.,Muhlenhoff, U.,Banci, L.,Lill, R.
Mitochondrial Bol1 and Bol3 function as assembly factors for specific iron-sulfur proteins.
Elife, 5:-, 2016

PubMed 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.

PubMed: 27532772
DOI: 10.7554/eLife.16673

Experimental method

SOLUTION NMR