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The regulation of chromatin structure is of paramount importance for a variety of fundamental nuclear processes, including gene expression, DNA repair, replication, and recombination. The ATP-dependent chromatin-remodelling factor ATRX (α thalassaemia/mental retardation X-linked) has emerged as a key player in each of these processes. Exciting recent developments suggest that ATRX plays a variety of key roles at tandem repeat sequences within the genome, including the deposition of a histone variant, prevention of replication fork stalling, and the suppression of a homologous recombination-based pathway of telomere maintenance. Here, we provide a mechanistic overview of the role of ATRX in each of these processes, and propose how they may be connected to give rise to seemingly disparate human diseases.

Original publication

DOI

10.1016/j.tibs.2013.06.011

Type

Journal article

Journal

Trends Biochem Sci

Publication Date

09/2013

Volume

38

Pages

461 - 466

Keywords

ATRX, DNA replication, G4-quadruplex DNA, alternative lengthening of telomeres, Chromatin, Chromatin Assembly and Disassembly, DNA Helicases, DNA Replication, Histones, Humans, Nuclear Proteins, Telomere, X-linked Nuclear Protein