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2iqc

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[[Image:2iqc.gif|left|200px]]
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{{Structure
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|GENE= FANCF ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
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{{STRUCTURE_2iqc| PDB=2iqc | SCENE= }}
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|RESOURCES=<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://www.ebi.ac.uk/pdbsum/2iqc PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=2iqc RCSB]</span>
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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'''
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[[Category: Lehmann, C.]]
[[Category: Lehmann, C.]]
[[Category: Stuckert, P.]]
[[Category: Stuckert, P.]]
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[[Category: complex subunit]]
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[[Category: Complex subunit]]
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[[Category: dna-damage]]
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[[Category: Dna-damage]]
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[[Category: fanconi]]
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[[Category: Fanconi]]
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[[Category: heat-like repeat]]
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[[Category: Heat-like repeat]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun May 4 07:46:20 2008''
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Revision as of 04:46, 4 May 2008

Image:2iqc.gif

Template:STRUCTURE 2iqc

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. 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 Page seeded by OCA on Sun May 4 07:46:20 2008

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