2iqc
From Proteopedia
(New page: 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 /> | + | [[Image:2iqc.gif|left|200px]]<br /><applet load="2iqc" size="350" color="white" frame="true" align="right" spinBox="true" |
| - | <applet load="2iqc" size=" | + | |
caption="2iqc, resolution 2.40Å" /> | caption="2iqc, resolution 2.40Å" /> | ||
'''Crystal structure of Human FancF Protein that Functions in the Assembly of a DNA Damage Signaling Complex'''<br /> | '''Crystal structure of Human FancF Protein that Functions in the Assembly of a DNA Damage Signaling Complex'''<br /> | ||
==Overview== | ==Overview== | ||
| - | Fanconi anemia (FA) is a rare autosomal recessive and X-linked chromosomal | + | 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. |
==Disease== | ==Disease== | ||
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==About this Structure== | ==About this Structure== | ||
| - | 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:// | + | 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 <scene name='pdbligand=HG:'>HG</scene> as [http://en.wikipedia.org/wiki/ligand ligand]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2IQC OCA]. |
==Reference== | ==Reference== | ||
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[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Single protein]] | [[Category: Single protein]] | ||
| - | [[Category: Andrea, A | + | [[Category: Andrea, A D.]] |
| - | [[Category: Ellenberger, T | + | [[Category: Ellenberger, T E.]] |
| - | [[Category: Gurtan, A | + | [[Category: Gurtan, A M.]] |
[[Category: Kowal, P.]] | [[Category: Kowal, P.]] | ||
[[Category: Lehmann, C.]] | [[Category: Lehmann, C.]] | ||
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[[Category: heat-like repeat]] | [[Category: heat-like repeat]] | ||
| - | ''Page seeded by [http:// | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 17:55:04 2008'' |
Revision as of 15:55, 21 February 2008
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Crystal structure of Human FancF Protein that Functions in the Assembly of a DNA Damage Signaling Complex
Contents |
Overview
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.
Disease
Known disease associated with this structure: Fanconi anemia, complementation group F OMIM:[603467]
About this Structure
2IQC is a Single protein structure of sequence from Homo sapiens with as ligand. Full crystallographic information is available from OCA.
Reference
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
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