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==Crystal structure of Human FancF Protein that Functions in the Assembly of a DNA Damage Signaling Complex==
==Crystal structure of Human FancF Protein that Functions in the Assembly of a DNA Damage Signaling Complex==
<StructureSection load='2iqc' size='340' side='right' caption='[[2iqc]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
<StructureSection load='2iqc' size='340' side='right'caption='[[2iqc]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[2iqc]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2IQC OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2IQC FirstGlance]. <br>
<table><tr><td colspan='2'>[[2iqc]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2IQC OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2IQC FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=HG:MERCURY+(II)+ION'>HG</scene></td></tr>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.4&#8491;</td></tr>
<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">FANCF ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=HG:MERCURY+(II)+ION'>HG</scene></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=2iqc FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2iqc OCA], [http://pdbe.org/2iqc PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2iqc RCSB], [http://www.ebi.ac.uk/pdbsum/2iqc PDBsum]</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=2iqc FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2iqc OCA], [https://pdbe.org/2iqc PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2iqc RCSB], [https://www.ebi.ac.uk/pdbsum/2iqc PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2iqc ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/FANCF_HUMAN FANCF_HUMAN]] Defects in FANCF are the cause of Fanconi anemia complementation group F (FANCF) [MIM:[http://omim.org/entry/603467 603467]]. A disorder affecting all bone marrow elements and resulting in anemia, leukopenia and thrombopenia. It is associated with cardiac, renal and limb malformations, dermal pigmentary changes, and a predisposition to the development of malignancies. At the cellular level it is associated with hypersensitivity to DNA-damaging agents, chromosomal instability (increased chromosome breakage) and defective DNA repair.<ref>PMID:10615118</ref>
[https://www.uniprot.org/uniprot/FANCF_HUMAN FANCF_HUMAN] Defects in FANCF are the cause of Fanconi anemia complementation group F (FANCF) [MIM:[https://omim.org/entry/603467 603467]. A disorder affecting all bone marrow elements and resulting in anemia, leukopenia and thrombopenia. It is associated with cardiac, renal and limb malformations, dermal pigmentary changes, and a predisposition to the development of malignancies. At the cellular level it is associated with hypersensitivity to DNA-damaging agents, chromosomal instability (increased chromosome breakage) and defective DNA repair.<ref>PMID:10615118</ref>  
== Function ==
== Function ==
[[http://www.uniprot.org/uniprot/FANCF_HUMAN FANCF_HUMAN]] DNA repair protein that may operate in a postreplication repair or a cell cycle checkpoint function. May be implicated in interstrand DNA cross-link repair and in the maintenance of normal chromosome stability (By similarity).  
[https://www.uniprot.org/uniprot/FANCF_HUMAN FANCF_HUMAN] DNA repair protein that may operate in a postreplication repair or a cell cycle checkpoint function. May be implicated in interstrand DNA cross-link repair and in the maintenance of normal chromosome stability (By similarity).
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
Fanconi anemia (FA) is a rare autosomal recessive and X-linked chromosomal instability disorder. At least eight FA proteins (FANCA, B, C, E, F, G, L, and M) form a nuclear core complex required for monoubiquitination of a downstream protein, FANCD2. The human FANCF protein reportedly functions as a molecular adaptor within the FA nuclear complex, bridging between the subcomplexes A:G and C:E. Our x-ray crystallographic studies of the C-terminal domain of FANCF reveal a helical repeat structure similar to the Cand1 regulator of the Cul1-Rbx1-Skp1-Fbox(Skp2) ubiquitin ligase complex. Two C-terminal loops of FANCF are essential for monoubiquitination of FANCD2 and normal cellular resistance to the DNA cross-linking agent mitomycin C. FANCF mutants bearing amino acid substitutions in this C-terminal surface fail to interact with other components of the FA complex, indicating that this surface is critical for the proper assembly of the FA core complex.
 
Structural determinants of human FANCF protein that function in the assembly of a DNA damage signaling complex.,Kowal P, Gurtan AM, Stuckert P, D'Andrea AD, Ellenberger T J Biol Chem. 2007 Jan 19;282(3):2047-55. Epub 2006 Nov 1. PMID:17082180<ref>PMID:17082180</ref>
 
From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 2iqc" style="background-color:#fffaf0;"></div>
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
[[Category: Human]]
[[Category: Homo sapiens]]
[[Category: Andrea, A D]]
[[Category: Large Structures]]
[[Category: Ellenberger, T E]]
[[Category: D'Andrea A]]
[[Category: Gurtan, A M]]
[[Category: Ellenberger TE]]
[[Category: Kowal, P]]
[[Category: Gurtan AM]]
[[Category: Lehmann, C]]
[[Category: Kowal P]]
[[Category: Stuckert, P]]
[[Category: Lehmann C]]
[[Category: Complex subunit]]
[[Category: Stuckert P]]
[[Category: Dna-damage]]
[[Category: Fanconi]]
[[Category: Heat-like repeat]]
[[Category: Protein binding]]
Retrieved from "https://proteopedia.org/2iqc"