This old version of Proteopedia is provided for student assignments while the new version is undergoing repairs. Content and edits done in this old version of Proteopedia after March 1, 2026 will eventually be lost when it is retired in about June of 2026.

Apply for new accounts at the new Proteopedia. Your logins will work in both the old and new versions.

2ncl

From Proteopedia

(Difference between revisions)

Jump to: navigation, search

Current revision

Solution structure of BOLA3 from Homo sapiens

Structural highlights

2ncl is a 1 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA. For a guided tour on the structure components use FirstGlance.
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

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

1 Structural highlights
2 Disease
3 Function
4 Publication Abstract from PubMed
5 References

Proteopedia Page Contributors and Editors (what is this?)

OCA

Retrieved from "http://52.214.119.220/wiki/index.php/2ncl"

Categories: Homo sapiens | Large Structures | Banci L | Ciofi-Baffoni S | Nasta V

@@ Line 1: / Line 1: @@
 ==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 ==
-<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>
+<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='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>
+</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>
-<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.
+[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;">
 == Publication Abstract from PubMed ==
@@ Line 22: / Line 23: @@
 __TOC__
 </StructureSection>
-[[Category: Human]]
+[[Category: Homo sapiens]]
 [[Category: Large Structures]]
-[[Category: Banci, L]]
+[[Category: Banci L]]
-[[Category: Ciofi-Baffoni, S]]
+[[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]]

2ncl

From Proteopedia

Current revision

Solution structure of BOLA3 from Homo sapiens

Structural highlights

Disease

Function

Publication Abstract from PubMed

References

Contents

Proteopedia Page Contributors and Editors (what is this?)

Views

Personal tools

Navigation

Search

Toolbox