Emerging Roles of ATRX in Cancer

Abstract

ATRX was identified over 20 years ago as the gene responsible for a rare developmental disorder characterized by α-thalassemia and intellectual disability. Similarities to the sucrose nonfermentable SNF2 type chromatin remodelers initially suggested a role in transcriptional regulation. However, over the last years, our knowledge of the epigenetic activities of ATRX has expanded steadily. Recent exciting discoveries have propelled ATRX into the limelight of chromatin and telomere biology, development and cancer research. This review summarizes recent breakthroughs in understanding ATRX function in heterochromatin structure, genome stability and its frequent dysregulation in a variety of cancers.

Keywords::

• alternative lengthening of telomeres
• ATRX
• cancer
• chromatin
• DAXX
• DNA replication
• G quadruplexes
• histone H3.3
• p53
• telomeres

Financial & competing interests disclosure

The authors greatfully acknowledge funding from the Cancer Research Society (CRS) and the Department of Oncology Catalyst Grant fund. LA Watson was funded by an NSERC fellowship. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Additional information

Funding

The authors greatfully acknowledge funding from the Cancer Research Society (CRS) and the Department of Oncology Catalyst Grant fund. LA Watson was funded by an NSERC fellowship. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. No writing assistance was utilized in the production of this manuscript.