Topic Completed: 1 February 2014

Minor changes: 27 August 2020

Copyright: 2003-2020,, Inc.

PubMed Search: ATRX[title]

Nat Pernick, M.D.
Page views in 2019: 3,712
Page views in 2020 to date: 3,128
Cite this page: Pernick N. ATDX (ATRX). website. Accessed September 26th, 2020.
Definition / general
  • Alpha-Thalassemia/intellectual Disability syndrome X-linked
  • Chromatin remodelling protein important in DNA replication, telomere stability, gene transcription, chromosome congression and cohesion during cell division (Epigenetics 2013;8:3)
  • Member of SWI/SNF protein family of DNA dependent ATPases
  • Is recruited to site of DNA damage; required for efficient checkpoint activation and faithful replication restart (J Biol Chem 2013;288:6342)
  • Death domain-associated protein (Daxx) cooperates with ATRX
  • Previously termed Alpha Thalassemia / Mental Retardation syndrome
Clinical features
Uses by pathologists
  • See clinical features above
Microscopic (histologic) images

Images hosted on other servers:


Pancreas: ALT+ tumors

Pancreas: MEN1
microadenoma and
pancreatic neuroendocrine

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