ATRX, a guardian of chromatin

ATRX (alpha-thalassemia mental retardation X-linked) is one of the most fre-quently mutated tumor suppressor genes in human cancers, especially in gli-oma, and recent findings indicate roles for ATRX in key molecular pathways, such as the regulation of chromatin state, gene expression, and DNA damage re-pair, placing ATRX as a central player in the maintenance of genome stability and function. This has led to new perspectives about the functional role of ATRX and its relationship with cancer. Here, we provide an overview of ATRX interactions and molecular functions and discuss the consequences of its impairment, in-cluding alternative lengthening of telomeres and therapeutic vulnerabilities that may be exploited in cancer cells.

Automated summary

ATRX is a frequently mutated tumor suppressor gene in human cancers, especially in glioma. Recent studies have revealed its important roles in key molecular pathways, such as the regulation of chromatin state, gene expression, and DNA damage repair, making it a central player in maintaining genome stability and function. This article provides an overview of ATRX interactions and molecular functions, as well as discussing the consequences of its impairment, including alternative lengthening of telomeres and therapeutic vulnerabilities in cancer cells.