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Background  The family of RecQ DNA helicases plays an important role in the maintenance of genomic integrity. Mutations in three of the five known RecQ family members in humans,  BLM ,  WRN  and  RecQ4 , lead to disorders that are characterized by predisposition to cancer and premature aging.    Methodology/Principal Findings  To address the  in vivo  functions of  Drosophila RecQ4  ( dRecQ4 ), we generated mutant alleles of  dRecQ4  using the targeted gene knock-out technique. Our data show that  dRecQ4  mutants are homozygous lethal with defects in DNA replication, cell cycle progression and cell proliferation. Two sets of experiments suggest that  dRecQ4  also plays a role in DNA double strand break repair. First, mutant animals exhibit sensitivity to gamma irradiation. Second, the efficiency of DsRed reconstitution via single strand annealing repair is significantly reduced in the  dRecQ4  mutant animals. Rescue experiments further show that both the N-terminal domain and the helicase domain are essential to dRecQ4 function  in vivo . The N-terminal domain is sufficient for the DNA repair function of dRecQ4.    Conclusions/Significance  Together, our results show that  dRecQ4  is an essential gene that plays an important role in not only DNA replication but also DNA repair and cell cycle progression  in vivo .

dRecQ4 Is Required for DNA Synthesis and Essential for Cell Proliferation in Drosophila