title: 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.
authors.affiliation: Departments of Paediatrics, Biochemistry & Oncology, University of Western Ontario, Victoria Research Laboratories, 800 Commissioners Road East, London, Canada.|Children's Health Research Institute, London, Canada.|Lawson Health Research Institute, London, Canada.
authors.forename: L Ashley
authors.initials: LA
authors.lastname: Watson
authors.affiliation: Departments of Paediatrics, Biochemistry & Oncology, University of Western Ontario, Victoria Research Laboratories, 800 Commissioners Road East, London, Canada.|Children's Health Research Institute, London, Canada.|Lawson Health Research Institute, London, Canada.
authors.forename: Hannah
authors.initials: H
authors.lastname: Goldberg
authors.affiliation: Departments of Paediatrics, Biochemistry & Oncology, University of Western Ontario, Victoria Research Laboratories, 800 Commissioners Road East, London, Canada.|Children's Health Research Institute, London, Canada.|Lawson Health Research Institute, London, Canada.
authors.forename: Nathalie G
authors.initials: NG
authors.lastname: Bérubé
mesh_terms: D048868:Adaptor Proteins, Signal Transducing / Q000378:metabolism; D000818:Animals; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000378:metabolism; D042002:Chromatin Assembly and Disassembly; D056970:Co-Repressor Proteins; D004265:DNA Helicases / Q000235:genetics* / Q000378:metabolism*; D054856:G-Quadruplexes; D015972:Gene Expression Regulation, Neoplastic; D020868:Gene Silencing; D042822:Genomic Instability; D006570:Heterochromatin / Q000235:genetics / Q000378:metabolism; D006801:Humans; D018832:Molecular Chaperones; D046912:Multiprotein Complexes / Q000378:metabolism; D009154:Mutation; D009369:Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000378:metabolism*; D009687:Nuclear Proteins / Q000235:genetics* / Q000378:metabolism*; D011485:Protein Binding; D059505:Telomere Homeostasis; D000075924:X-linked Nuclear Protein
chemical_list: D048868:Adaptor Proteins, Signal Transducing; D056970:Co-Repressor Proteins; C106985:DAXX protein, human; D006570:Heterochromatin; D018832:Molecular Chaperones; D046912:Multiprotein Complexes; D009687:Nuclear Proteins; D004265:DNA Helicases; C092332:ATRX protein, human; D000075924:X-linked Nuclear Protein
keywords: ATRX; DAXX; DNA replication; G quadruplexes; alternative lengthening of telomeres; cancer; chromatin; histone H3.3; p53; telomeres