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- W3186303608 endingPage "1112" @default.
- W3186303608 startingPage "1112" @default.
- W3186303608 abstract "Telomere maintenance plays important roles in genome stability and cell proliferation. Tumor cells acquire replicative immortality by activating a telomere-maintenance mechanism (TMM), either telomerase, a reverse transcriptase, or the alternative lengthening of telomeres (ALT) mechanism. Recent advances in the genetic and molecular characterization of TMM revealed that telomerase activation and ALT define distinct neuroblastoma (NB) subgroups with adverse outcomes, and represent promising therapeutic targets in high-risk neuroblastoma (HRNB), an aggressive childhood solid tumor that accounts for 15% of all pediatric-cancer deaths. Patients with HRNB frequently present with widely metastatic disease, with tumors harboring recurrent genetic aberrations (MYCN amplification, TERT rearrangements, and ATRX mutations), which are mutually exclusive and capable of promoting TMM. This review provides recent insights into our understanding of TMM in NB tumors, and highlights emerging therapeutic strategies as potential treatments for telomerase- and ALT-positive tumors." @default.
- W3186303608 created "2021-08-02" @default.
- W3186303608 creator A5064427269 @default.
- W3186303608 creator A5091595230 @default.
- W3186303608 date "2021-07-28" @default.
- W3186303608 modified "2023-10-16" @default.
- W3186303608 title "How Do Telomere Abnormalities Regulate the Biology of Neuroblastoma?" @default.
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