Structure of the FANCI-FANCD2 complex: Insights into the Fanconi anemia DNA repair pathway Journal Article


Authors: Joo, W.; Xu, G.; Persky, N. S.; Smogorzewska, A.; Rudge, D. G.; Buzovetsky, O.; Elledge, S. J.; Pavletich, N. P.
Article Title: Structure of the FANCI-FANCD2 complex: Insights into the Fanconi anemia DNA repair pathway
Abstract: Fanconi anemia is a cancer predisposition syndrome caused by defects in the repair of DNA interstrand cross-links (ICLs). Central to this pathway is the Fanconi anemia I - Fanconi anemia D2 (FANCI-FANCD2) (ID) complex, which is activated by DNA damage - induced phosphorylation and monoubiquitination. The 3.4 angstrom crystal structure of the ∼300 kilodalton ID complex reveals that monoubiquitination and regulatory phosphorylation sites map to the I-D interface, suggesting that they occur on monomeric proteins or an opened-up complex and that they may serve to stabilize I-D heterodimerization. The 7.8 angstrom electron-density map of FANCI-DNA crystals and in vitro data show that each protein has binding sites for both single- and double-stranded DNA, suggesting that the ID complex recognizes DNA structures that result from the encounter of replication forks with an ICL.
Journal Title: Science
Volume: 333
Issue: 6040
ISSN: 0036-8075
Publisher: American Association for the Advancement of Science  
Date Published: 2011-07-15
Start Page: 312
End Page: 316
Language: English
DOI: 10.1126/science.1205805
PROVIDER: scopus
PUBMED: 21764741
PMCID: PMC3310437
DOI/URL:
Notes: --- - "Export Date: 17 August 2011" - "CODEN: SCIEA" - "Source: Scopus"
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MSK Authors
  1. Woo S Joo
    4 Joo
  2. Derek Rudge
    2 Rudge
  3. Nicole Sylvia Persky
    2 Persky
  4. Guozhou Xu
    2 Xu