2iqc: Difference between revisions

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[[Image:2iqc.gif|left|200px]]
{{Seed}}
[[Image:2iqc.png|left|200px]]


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{{STRUCTURE_2iqc|  PDB=2iqc  |  SCENE=  }}  
{{STRUCTURE_2iqc|  PDB=2iqc  |  SCENE=  }}  


'''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===




==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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