A high prevalence of myeloid malignancies in progeria with Werner syndrome is associated with p53 insufficiency

Werner syndrome (WS) is a progeroid syndrome characterized by the early onset of aging-related symptoms [1,2]. It is caused by mutations in the WRN gene on 8p12, which encodes the RecQ type DNA helicase. The WRN helicase belongs to a DEAH box-containing RecQ family of helicases and has a high degree of helicase activity for the unwinding of unusual DNA structures, such as G4 quadruplex sequences and four-way junctions that resemble intermediates of DNA repair and telomere maintenance. It has been hypothesized that WRN plays a role in the resolution of potentially damaging, complex DNA structures that were accidentally formed during various DNA transactions, including replication, recombination, repair, and transcription [2].

Source: Experimental Hematology - February 28, 2022 Category: Hematology Authors: Hisaya Kato, Yoshiro Maezawa, Dai Nishijima, Eisuke Iwamoto, June Takeda, Takashi Kanamori, Masaya Yamaga, Tatsuzo Mishina, Yusuke Takeda, Shintaro Izumi, Yutaro Hino, Hiroyuki Nishi, Jun Ishiko, Masahiro Takeuchi, Hiyori Kaneko, Masaya Koshizaka, Naoya M Tags: Brief Communication Source Type: research