Alternative lengthening of telomeres phenotype and loss of ATRX expression in sarcomas (Review)

  • Authors:
    • Xiaolei Ren
    • Chao Tu
    • Zhenchu Tang
    • Ruofei Ma
    • Zhihong Li
  • View Affiliations

  • Published online on: March 22, 2018     https://doi.org/10.3892/ol.2018.8318
  • Pages: 7489-7496
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Abstract

Sarcoma is a rare and heterogeneous type of cancer with an early mean onset age and a poor prognosis. However, its genetic basis remains unclear. A series of recent genomic studies in sarcomas have identified the occurrence of mutations in the α‑thalassemia/mental retardation syndrome X‑linked (ATRX) gene. The ATRX protein belongs to the SWI/SNF family of chromatin remodeling proteins, which are frequently associated with α‑thalassemia syndrome. Cancer cells depend on telomerase or the alternative lengthening of telomeres (ALT) pathway to overcome replicative programmed mortality. Loss of ATRX is associated with ALT in sarcoma. In the present review, recent whole genome and/or whole exome genomic studies are summarized. In addition ATRX immunohistochemistry and ALT fluorescence in situ hybridization in sarcomas of various subtypes and at diverse sites, including osteosarcoma, leiomyosarcoma, liposarcoma, angiosarcoma and chondrosarcoma are evaluated. The present review involves certain studies associated with the molecular mechanisms underlying the loss of ATRX controlling the activation of ALT in sarcomas. Identification of the loss of ATRX and ALT in sarcomas may provide novel methods for the treatment of aggressive sarcomas.
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May-2018
Volume 15 Issue 5

Print ISSN: 1792-1074
Online ISSN:1792-1082

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Spandidos Publications style
Ren X, Tu C, Tang Z, Ma R and Li Z: Alternative lengthening of telomeres phenotype and loss of ATRX expression in sarcomas (Review). Oncol Lett 15: 7489-7496, 2018
APA
Ren, X., Tu, C., Tang, Z., Ma, R., & Li, Z. (2018). Alternative lengthening of telomeres phenotype and loss of ATRX expression in sarcomas (Review). Oncology Letters, 15, 7489-7496. https://doi.org/10.3892/ol.2018.8318
MLA
Ren, X., Tu, C., Tang, Z., Ma, R., Li, Z."Alternative lengthening of telomeres phenotype and loss of ATRX expression in sarcomas (Review)". Oncology Letters 15.5 (2018): 7489-7496.
Chicago
Ren, X., Tu, C., Tang, Z., Ma, R., Li, Z."Alternative lengthening of telomeres phenotype and loss of ATRX expression in sarcomas (Review)". Oncology Letters 15, no. 5 (2018): 7489-7496. https://doi.org/10.3892/ol.2018.8318