View source for 6nzu


==Structure of the human frataxin-bound iron-sulfur cluster assembly complex==
<SX load='6nzu' size='340' side='right' viewer='molstar' caption='[[6nzu]], [[Resolution|resolution]] 3.20&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[6nzu]] is a 10 chain structure with sequence from [https://en.wikipedia.org/wiki/Escherichia_coli Escherichia coli] and [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6NZU OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6NZU FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3.2&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=8Q1:S-[2-({N-[(2R)-2-hydroxy-3,3-dimethyl-4-(phosphonooxy)butanoyl]-beta-alanyl}amino)ethyl]+dodecanethioate'>8Q1</scene>, <scene name='pdbligand=PLP:PYRIDOXAL-5-PHOSPHATE'>PLP</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></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=6nzu FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6nzu OCA], [https://pdbe.org/6nzu PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6nzu RCSB], [https://www.ebi.ac.uk/pdbsum/6nzu PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6nzu ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/NFS1_HUMAN NFS1_HUMAN] Severe neonatal lactic acidosis due to NFS1-ISD11 complex deficiency. 
== Function ==
[https://www.uniprot.org/uniprot/NFS1_HUMAN NFS1_HUMAN] Catalyzes the removal of elemental sulfur from cysteine to produce alanine. It supplies the inorganic sulfur for iron-sulfur (Fe-S) clusters. May be involved in the biosynthesis of molybdenum cofactor.<ref>PMID:18650437</ref> 
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== Publication Abstract from PubMed ==
The core machinery for de novo biosynthesis of iron-sulfur clusters (ISC), located in the mitochondria matrix, is a five-protein complex containing the cysteine desulfurase NFS1 that is activated by frataxin (FXN), scaffold protein ISCU, accessory protein ISD11, and acyl-carrier protein ACP. Deficiency in FXN leads to the loss-of-function neurodegenerative disorder Friedreich's ataxia (FRDA). Here the 3.2 A resolution cryo-electron microscopy structure of the FXN-bound active human complex, containing two copies of the NFS1-ISD11-ACP-ISCU-FXN hetero-pentamer, delineates the interactions of FXN with other component proteins of the complex. FXN binds at the interface of two NFS1 and one ISCU subunits, modifying the local environment of a bound zinc ion that would otherwise inhibit NFS1 activity in complexes without FXN. Our structure reveals how FXN facilitates ISC production through stabilizing key loop conformations of NFS1 and ISCU at the protein-protein interfaces, and suggests how FRDA clinical mutations affect complex formation and FXN activation.

Structure of the human frataxin-bound iron-sulfur cluster assembly complex provides insight into its activation mechanism.,Fox NG, Yu X, Feng X, Bailey HJ, Martelli A, Nabhan JF, Strain-Damerell C, Bulawa C, Yue WW, Han S Nat Commun. 2019 May 17;10(1):2210. doi: 10.1038/s41467-019-09989-y. PMID:31101807<ref>PMID:31101807</ref>

From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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<div class="pdbe-citations 6nzu" style="background-color:#fffaf0;"></div>

==See Also==
*[[Acyl carrier protein 3D structures|Acyl carrier protein 3D structures]]
*[[Cysteine desulfurase 3D structures|Cysteine desulfurase 3D structures]]
*[[Frataxin 3D Structures|Frataxin 3D Structures]]
== References ==
<references/>
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</SX>
[[Category: Escherichia coli]]
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Alain M]]
[[Category: Christine B]]
[[Category: Claire SD]]
[[Category: Fox NG]]
[[Category: Han S]]
[[Category: Joseph N]]
[[Category: Xidong F]]
[[Category: Yu X]]
[[Category: Yue WW]]