rdf:type |
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lifeskim:mentions |
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pubmed:issue |
6040
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pubmed:dateCreated |
2011-7-18
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pubmed:databankReference |
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pubmed:abstractText |
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.
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pubmed:grant |
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Jul
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pubmed:issn |
1095-9203
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pubmed:author |
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pubmed:issnType |
Electronic
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pubmed:day |
15
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pubmed:volume |
333
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
312-6
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pubmed:meshHeading |
pubmed-meshheading:21764741-Amino Acid Sequence,
pubmed-meshheading:21764741-Animals,
pubmed-meshheading:21764741-Binding Sites,
pubmed-meshheading:21764741-Crystallography, X-Ray,
pubmed-meshheading:21764741-DNA,
pubmed-meshheading:21764741-DNA, Single-Stranded,
pubmed-meshheading:21764741-DNA Repair,
pubmed-meshheading:21764741-Fanconi Anemia,
pubmed-meshheading:21764741-Fanconi Anemia Complementation Group D2 Protein,
pubmed-meshheading:21764741-Fanconi Anemia Complementation Group Proteins,
pubmed-meshheading:21764741-Hydrophobic and Hydrophilic Interactions,
pubmed-meshheading:21764741-Mice,
pubmed-meshheading:21764741-Models, Molecular,
pubmed-meshheading:21764741-Molecular Sequence Data,
pubmed-meshheading:21764741-Phosphorylation,
pubmed-meshheading:21764741-Protein Binding,
pubmed-meshheading:21764741-Protein Conformation,
pubmed-meshheading:21764741-Protein Folding,
pubmed-meshheading:21764741-Protein Structure, Secondary,
pubmed-meshheading:21764741-Protein Structure, Tertiary,
pubmed-meshheading:21764741-Static Electricity,
pubmed-meshheading:21764741-Ubiquitin,
pubmed-meshheading:21764741-Ubiquitination
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pubmed:year |
2011
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pubmed:articleTitle |
Structure of the FANCI-FANCD2 complex: insights into the Fanconi anemia DNA repair pathway.
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pubmed:affiliation |
Structural Biology Program, Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't,
Research Support, N.I.H., Extramural
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