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2iqc

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{{STRUCTURE_2iqc| PDB=2iqc | SCENE= }}
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'''Crystal structure of Human FancF Protein that Functions in the Assembly of a DNA Damage Signaling Complex'''
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===Crystal structure of Human FancF Protein that Functions in the Assembly of a DNA Damage Signaling Complex===
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==Overview==
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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.
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==Disease==
==Disease==
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[[Category: Fanconi]]
[[Category: Fanconi]]
[[Category: Heat-like repeat]]
[[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 21:10, 28 July 2008

Image:2iqc.png

Template:STRUCTURE 2iqc

Contents

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

Template:ABSTRACT PUBMED 17082180

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

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