View source for 2ncl


==Solution structure of BOLA3 from Homo sapiens==
<StructureSection load='2ncl' size='340' side='right'caption='[[2ncl]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[2ncl]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. 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='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">BOLA3 ([https://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</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>
== 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. 
<div style="background-color:#fffaf0;">
== 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<ref>PMID:27532772</ref>

From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 2ncl" style="background-color:#fffaf0;"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Human]]
[[Category: Large Structures]]
[[Category: Banci, L]]
[[Category: Ciofi-Baffoni, S]]
[[Category: Nasta, V]]
[[Category: Class ii kh-like fold]]
[[Category: Fe/s protein biogenesis]]
[[Category: Mitochondrial protein]]
[[Category: Protein binding]]