Research ArticleCancer

ATRX loss promotes tumor growth and impairs nonhomologous end joining DNA repair in glioma

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Science Translational Medicine  02 Mar 2016:
Vol. 8, Issue 328, pp. 328ra28
DOI: 10.1126/scitranslmed.aac8228

Aggressive gliomas’ Achilles’ heel

ATRX is a protein that is often mutated in glioma, a lethal and relatively common brain tumor. Koschmann et al. developed a mouse model of ATRX-deficient glioma and discovered that these tumors grow more aggressively than their counterparts with wild-type ATRX. The reason this happens is that the loss of ATRX impairs DNA repair, resulting in genetically unstable tumors that can accumulate oncogenic mutations more quickly. However, because of their DNA repair defect, these tumors also proved to be more sensitive to treatments that damage the DNA, such as radiation and some types of chemotherapy. Consistent with these findings, the presence of ATRX mutation correlated with better outcomes in patients, because these tumors were more susceptible to treatment.

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