RECQL5 and BLM exhibit divergent functions in cells defective for the Fanconi anemia pathway
RECQL5 and BLM exhibit divergent functions in cells defective for the Fanconi anemia pathway
Fanconi anemia (FA) patients exhibit bone marrow failure, developmental defects and cancer. The FA pathway maintains chromosomal stability in concert with replication fork maintenance and DNA double strand break (DSB) repair pathways including RAD51-mediated homologous recombination (HR). RAD51 is a recombinase that maintains replication forks and repairs DSBs, but also rearranges chromosomes. Two RecQ helicases, RECQL5 and Bloom syndrome mutated (BLM) suppress HR through nonredundant mechanisms. Here we test the impact deletion of RECQL5 and BLM has on mouse embryonic stem (ES) cells deleted for FANCB, a member of the FA core complex. We show that RECQL5, but not BLM, conferred resistance to mitomycin C (MMC, an interstrand crosslinker) and camptothecin (CPT, a type 1 topoisomerase inhibitor) in FANCB-defective cells. RECQL5 suppressed, while BLM caused, breaks and radials in FANCB-deleted cells exposed to CPT or MMC, respectively. RECQL5 protected the nascent replication strand from MRE11-mediated degradation and restarted stressed replication forks in a manner additive to FANCB. By contrast BLM restarted, but did not protect, replication forks in a manner epistatic to FANCB. RECQL5 also lowered RAD51 levels in FANCB-deleted cells at stressed replication sites implicating a rearrangement avoidance mechanism. Thus, RECQL5 and BLM impact FANCB-defective cells differently in response to replication stress with relevance to chemotherapeutic regimes.
- Case Western Reserve University United States
- The University of Texas System United States
- The University of Texas at San Antonio United States
- The University of Texas Health Science Center at San Antonio United States
- The University of Texas Health Science Center at Houston United States
DNA Replication, DNA Repair, RecQ Helicases, Genome Integrity, Repair and Replication, Fanconi Anemia Complementation Group Proteins, Mice, Animals, DNA Breaks, Double-Stranded, Cells, Cultured, Gene Deletion
DNA Replication, DNA Repair, RecQ Helicases, Genome Integrity, Repair and Replication, Fanconi Anemia Complementation Group Proteins, Mice, Animals, DNA Breaks, Double-Stranded, Cells, Cultured, Gene Deletion
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