View source for 6geu


==Crystal structure of the C230A mutant of human IBA57==
<StructureSection load='6geu' size='340' side='right'caption='[[6geu]], [[Resolution|resolution]] 1.55&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[6geu]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6GEU OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6GEU FirstGlance]. <br>
</td></tr><tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[5oli|5oli]], [[6esr|6esr]], [[6qe3|6qe3]], [[6qe4|6qe4]]</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=6geu FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6geu OCA], [http://pdbe.org/6geu PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6geu RCSB], [http://www.ebi.ac.uk/pdbsum/6geu PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6geu ProSAT]</span></td></tr>
</table>
== Disease ==
[[http://www.uniprot.org/uniprot/CAF17_HUMAN CAF17_HUMAN]] Hypotonia-cerebral atrophy-hyperglycinemia syndrome. The disease is caused by mutations affecting the gene represented in this entry.  The disease is caused by mutations affecting the gene represented in this entry. 
== Function ==
[[http://www.uniprot.org/uniprot/CAF17_HUMAN CAF17_HUMAN]] Involved in the maturation of mitochondrial 4Fe-4S proteins functioning late in the iron-sulfur cluster assembly pathway.<ref>PMID:23462291</ref>  
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
The maturation of mitochondrial iron-sulfur proteins requires a complex protein machinery. Human IBA57 protein was proposed to act in a late phase of this machinery, along with GLRX5, ISCA1, and ISCA2. However, a molecular picture on how these proteins cooperate is not defined yet. We show here that IBA57 forms a heterodimeric complex with ISCA2 by bridging a [2Fe-2S] cluster, that [2Fe-2S] cluster binding is absolutely required to promote the complex formation, and that the cysteine of the conserved motif characterizing IBA57 protein family and the three conserved cysteines of the ISCA protein family act as cluster ligands. The [2Fe-2S] heterodimeric complex is the final product when IBA57 is either exposed to [2Fe-2S] ISCA2 or in the presence of [2Fe-2S] GLRX5 and apo ISCA2. We also find that the [2Fe-2S] ISCA2-IBA57 complex is resistant to highly oxidative environments and is capable of reactivating apo aconitase in vitro. Collectively, our data delinate a [2Fe-2S] cluster transfer pathway involving three partner proteins of the mitochondrial ISC machinery, that is, GLRX5, ISCA2 and IBA57, which leads to the formation of a [2Fe-2S] ISCA2-IBA57 complex.

IBA57 Recruits ISCA2 to Form a [2Fe-2S] Cluster-Mediated Complex.,Gourdoupis S, Nasta V, Calderone V, Ciofi-Baffoni S, Banci L J Am Chem Soc. 2018 Oct 31;140(43):14401-14412. doi: 10.1021/jacs.8b09061. Epub, 2018 Oct 17. PMID:30269484<ref>PMID:30269484</ref>

From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 6geu" style="background-color:#fffaf0;"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Large Structures]]
[[Category: Banci, L]]
[[Category: Calderone, V]]
[[Category: Ciofi-Baffoni, S]]
[[Category: Gourdoupis, S]]
[[Category: Nasta, V]]
[[Category: Fe-s protein biogenesis]]
[[Category: Infantile leukodystrophy]]
[[Category: Mitochondrial protein]]
[[Category: Protein binding]]