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New page: left|200px<br /> <applet load="2iqc" size="450" color="white" frame="true" align="right" spinBox="true" caption="2iqc, resolution 2.40Å" /> '''Crystal structure o...
 
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[[Image:2iqc.gif|left|200px]]<br />
<applet load="2iqc" size="450" color="white" frame="true" align="right" spinBox="true"
caption="2iqc, resolution 2.40&Aring;" />
'''Crystal structure of Human FancF Protein that Functions in the Assembly of a DNA Damage Signaling Complex'''<br />


==Overview==
==Crystal structure of Human FancF Protein that Functions in the Assembly of a DNA Damage Signaling Complex==
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.
<StructureSection load='2iqc' size='340' side='right'caption='[[2iqc]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
 
== Structural highlights ==
==Disease==
<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>
Known disease associated with this structure: Fanconi anemia, complementation group F OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=603467 603467]]
</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='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>
==About this Structure==
<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>
2IQC is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] with HG as [http://en.wikipedia.org/wiki/ligand ligand]. Full crystallographic information is available from [http://ispc.weizmann.ac.il/oca-bin/ocashort?id=2IQC OCA].
</table>
 
== Disease ==
==Reference==
[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>
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:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=17082180 17082180]
== Function ==
[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).
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Single protein]]
[[Category: Large Structures]]
[[Category: Andrea, A.D.]]
[[Category: D'Andrea A]]
[[Category: Ellenberger, T.E.]]
[[Category: Ellenberger TE]]
[[Category: Gurtan, A.M.]]
[[Category: Gurtan AM]]
[[Category: Kowal, P.]]
[[Category: Kowal P]]
[[Category: Lehmann, C.]]
[[Category: Lehmann C]]
[[Category: Stuckert, P.]]
[[Category: Stuckert P]]
[[Category: HG]]
[[Category: complex subunit]]
[[Category: dna-damage]]
[[Category: fanconi]]
[[Category: heat-like repeat]]
 
''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 22:46:59 2007''

Latest revision as of 09:04, 21 February 2024

Crystal structure of Human FancF Protein that Functions in the Assembly of a DNA Damage Signaling Complex

2iqc, resolution 2.40Å

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