Journal
GENES & DEVELOPMENT
Volume 18, Issue 16, Pages 1958-1963Publisher
COLD SPRING HARBOR LAB PRESS, PUBLICATIONS DEPT
DOI: 10.1101/gad.1196104
Keywords
Fanconi anemia; FANCD2; cancer susceptibility; check-point; replication stress
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Funding
- NHLBI NIH HHS [P50 HL054785, R01 HL052725, P01HL54785, R01HL52725] Funding Source: Medline
- NIDDK NIH HHS [R01 DK043889, R01DK43889] Funding Source: Medline
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Fanconi anemia (FA) is a multigenic autosomal recessive cancer susceptibility syndrome. The FA pathway regulates the monoubiquitination of FANCD2 and the assembly of damage-associated FANCD2 nuclear foci. How FANCD2 monoubiquitination is coupled to the DNA-damage response has remained undetermined. Here, we demonstrate that the ATR checkpoint kinase and RPA1 are required for efficient FANCD2 monoubiquitination. Deficiency of ATR function, either in Seckel syndrome, which clinically resembles Fanconi anemia, or by siRNA silencing, results in the formation of radial chromosomes in response to the DNA cross-linker, mitomycin C (MMC), thus mimicking the chromosome instability of FA cells.
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