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| | ==Solution structure of BOLA3 from Homo sapiens== | | ==Solution structure of BOLA3 from Homo sapiens== |
| - | <StructureSection load='2ncl' size='340' side='right' caption='[[2ncl]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''> | + | <StructureSection load='2ncl' size='340' side='right'caption='[[2ncl]]' scene=''> |
| | == Structural highlights == | | == Structural highlights == |
| - | <table><tr><td colspan='2'>[[2ncl]] is a 1 chain structure. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2NCL OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2NCL FirstGlance]. <br> | + | <table><tr><td colspan='2'>[[2ncl]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2NCL OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2NCL FirstGlance]. <br> |
| - | </td></tr><tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2ncl FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2ncl OCA], [http://pdbe.org/2ncl PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2ncl RCSB], [http://www.ebi.ac.uk/pdbsum/2ncl PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2ncl ProSAT]</span></td></tr> | + | </td></tr><tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2ncl FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2ncl OCA], [https://pdbe.org/2ncl PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2ncl RCSB], [https://www.ebi.ac.uk/pdbsum/2ncl PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2ncl ProSAT]</span></td></tr> |
| | </table> | | </table> |
| | == Disease == | | == Disease == |
| - | [[http://www.uniprot.org/uniprot/BOLA3_HUMAN BOLA3_HUMAN]] Fatal multiple mitochondrial dysfunction syndrome type 2. The disease is caused by mutations affecting the gene represented in this entry. | + | [https://www.uniprot.org/uniprot/BOLA3_HUMAN BOLA3_HUMAN] Fatal multiple mitochondrial dysfunction syndrome type 2. The disease is caused by mutations affecting the gene represented in this entry. |
| | + | == Function == |
| | + | [https://www.uniprot.org/uniprot/BOLA3_HUMAN BOLA3_HUMAN] |
| | <div style="background-color:#fffaf0;"> | | <div style="background-color:#fffaf0;"> |
| | == Publication Abstract from PubMed == | | == Publication Abstract from PubMed == |
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| | __TOC__ | | __TOC__ |
| | </StructureSection> | | </StructureSection> |
| - | [[Category: Banci, L]] | + | [[Category: Homo sapiens]] |
| - | [[Category: Ciofi-Baffoni, S]] | + | [[Category: Large Structures]] |
| - | [[Category: Nasta, V]] | + | [[Category: Banci L]] |
| - | [[Category: Class ii kh-like fold]] | + | [[Category: Ciofi-Baffoni S]] |
| - | [[Category: Fe/s protein biogenesis]] | + | [[Category: Nasta V]] |
| - | [[Category: Mitochondrial protein]]
| + | |
| - | [[Category: Protein binding]]
| + | |
| Structural highlights
Disease
BOLA3_HUMAN Fatal multiple mitochondrial dysfunction syndrome type 2. The disease is caused by mutations affecting the gene represented in this entry.
Function
BOLA3_HUMAN
Publication Abstract from PubMed
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.
Mitochondrial Bol1 and Bol3 function as assembly factors for specific iron-sulfur proteins.,Uzarska MA, Nasta V, Weiler BD, Spantgar F, Ciofi-Baffoni S, Saviello MR, Gonnelli L, Muhlenhoff U, Banci L, Lill R Elife. 2016 Aug 17;5. pii: e16673. doi: 10.7554/eLife.16673. PMID:27532772[1]
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
References
- ↑ Uzarska MA, Nasta V, Weiler BD, Spantgar F, Ciofi-Baffoni S, Saviello MR, Gonnelli L, Muhlenhoff U, Banci L, Lill R. Mitochondrial Bol1 and Bol3 function as assembly factors for specific iron-sulfur proteins. Elife. 2016 Aug 17;5. pii: e16673. doi: 10.7554/eLife.16673. PMID:27532772 doi:http://dx.doi.org/10.7554/eLife.16673
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