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| | ==Solution structure of BOLA1 from Homo sapiens== | | ==Solution structure of BOLA1 from Homo sapiens== |
| - | <StructureSection load='5lci' size='340' side='right'caption='[[5lci]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''> | + | <StructureSection load='5lci' size='340' side='right'caption='[[5lci]]' scene=''> |
| | == Structural highlights == | | == Structural highlights == |
| - | <table><tr><td colspan='2'>[[5lci]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5LCI OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5LCI FirstGlance]. <br> | + | <table><tr><td colspan='2'>[[5lci]] 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=5LCI OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=5LCI FirstGlance]. <br> |
| - | </td></tr><tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">BOLA1, CGI-143 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr> | + | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</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=5lci FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5lci OCA], [http://pdbe.org/5lci PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5lci RCSB], [http://www.ebi.ac.uk/pdbsum/5lci PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5lci ProSAT]</span></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=5lci FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5lci OCA], [https://pdbe.org/5lci PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=5lci RCSB], [https://www.ebi.ac.uk/pdbsum/5lci PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=5lci ProSAT]</span></td></tr> |
| | </table> | | </table> |
| | + | == Function == |
| | + | [https://www.uniprot.org/uniprot/BOLA1_HUMAN BOLA1_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: Human]] | + | [[Category: Homo sapiens]] |
| | [[Category: Large Structures]] | | [[Category: Large Structures]] |
| - | [[Category: Baffoni, S Ciofi]] | + | [[Category: Banci L]] |
| - | [[Category: Banci, L]] | + | [[Category: Ciofi Baffoni S]] |
| - | [[Category: Nasta, V]] | + | [[Category: Nasta V]] |
| - | [[Category: Class ii kh-like fold]]
| + | |
| - | [[Category: Fe/s protein biogenesis]]
| + | |
| - | [[Category: Mitochondrial protein]]
| + | |
| - | [[Category: Protein binding]]
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| Structural highlights
Function
BOLA1_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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