Structure of the FANCI-FANCD2 complex: insights into the Fanconi anemia DNA repair pathway.
Science
; 333(6040): 312-6, 2011 Jul 15.
Article
in En
| MEDLINE
| ID: mdl-21764741
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
DNA Repair
/
Fanconi Anemia Complementation Group Proteins
/
Fanconi Anemia Complementation Group D2 Protein
Type of study:
Prognostic_studies
Limits:
Animals
Language:
En
Journal:
Science
Year:
2011
Document type:
Article
Affiliation country:
Estados Unidos