2ncl: Difference between revisions
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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 | <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 [ | <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'>[ | </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 == | ||
[ | [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: | [[Category: Homo sapiens]] | ||
[[Category: | [[Category: Large Structures]] | ||
[[Category: | [[Category: Banci L]] | ||
[[Category: | [[Category: Ciofi-Baffoni S]] | ||
[[Category: | [[Category: Nasta V]] | ||
Latest revision as of 13:27, 15 March 2023
Solution structure of BOLA3 from Homo sapiensSolution structure of BOLA3 from Homo sapiens
Structural highlights
DiseaseBOLA3_HUMAN Fatal multiple mitochondrial dysfunction syndrome type 2. The disease is caused by mutations affecting the gene represented in this entry. FunctionPublication Abstract from PubMedAssembly 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
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