The Chromatin Remodeler ATRX: Role and Mechanism in Biology and Cancer

The alpha-thalassemia mental retardation X-linked (ATRX) syndrome protein is a chromatin remodeling protein that primarily promotes the deposit of H3.3 histone variants in the telomere area. ATRX mutations not only cause ATRX syndrome but also influence development and promote cancer. The primary mo...

Full description

Bibliographic Details
Main Authors: Ying Pang, Xu Chen, Tongjie Ji, Meng Cheng, Rui Wang, Chunyu Zhang, Min Liu, Jing Zhang, Chunlong Zhong
Format: Article
Language:English
Published: MDPI AG 2023-04-01
Series:Cancers
Subjects:
Online Access:https://www.mdpi.com/2072-6694/15/8/2228
_version_ 1797606107866202112
author Ying Pang
Xu Chen
Tongjie Ji
Meng Cheng
Rui Wang
Chunyu Zhang
Min Liu
Jing Zhang
Chunlong Zhong
author_facet Ying Pang
Xu Chen
Tongjie Ji
Meng Cheng
Rui Wang
Chunyu Zhang
Min Liu
Jing Zhang
Chunlong Zhong
author_sort Ying Pang
collection DOAJ
description The alpha-thalassemia mental retardation X-linked (ATRX) syndrome protein is a chromatin remodeling protein that primarily promotes the deposit of H3.3 histone variants in the telomere area. ATRX mutations not only cause ATRX syndrome but also influence development and promote cancer. The primary molecular characteristics of ATRX, including its molecular structures and normal and malignant biological roles, are reviewed in this article. We discuss the role of ATRX in its interactions with the histone variant H3.3, chromatin remodeling, DNA damage response, replication stress, and cancers, particularly gliomas, neuroblastomas, and pancreatic neuroendocrine tumors. ATRX is implicated in several important cellular processes and serves a crucial function in regulating gene expression and genomic integrity throughout embryogenesis. However, the nature of its involvement in the growth and development of cancer remains unknown. As mechanistic and molecular investigations on ATRX disclose its essential functions in cancer, customized therapies targeting ATRX will become accessible.
first_indexed 2024-03-11T05:10:38Z
format Article
id doaj.art-f814ec908e284ee68c39a0865d857d14
institution Directory Open Access Journal
issn 2072-6694
language English
last_indexed 2024-03-11T05:10:38Z
publishDate 2023-04-01
publisher MDPI AG
record_format Article
series Cancers
spelling doaj.art-f814ec908e284ee68c39a0865d857d142023-11-17T18:38:05ZengMDPI AGCancers2072-66942023-04-01158222810.3390/cancers15082228The Chromatin Remodeler ATRX: Role and Mechanism in Biology and CancerYing Pang0Xu Chen1Tongjie Ji2Meng Cheng3Rui Wang4Chunyu Zhang5Min Liu6Jing Zhang7Chunlong Zhong8Department of Neurosurgery, Shanghai East Hospital, School of Medicine, Tongji University, 150 Jimo Road, Shanghai 200120, ChinaDepartment of Neurosurgery, Shanghai East Hospital, School of Medicine, Tongji University, 150 Jimo Road, Shanghai 200120, ChinaDepartment of Neurosurgery, Shanghai East Hospital, School of Medicine, Tongji University, 150 Jimo Road, Shanghai 200120, ChinaDepartment of Neurosurgery, Shanghai East Hospital, School of Medicine, Tongji University, 150 Jimo Road, Shanghai 200120, ChinaDepartment of Neurosurgery, Shanghai East Hospital, School of Medicine, Tongji University, 150 Jimo Road, Shanghai 200120, ChinaDepartment of Neurosurgery, Shanghai East Hospital, School of Medicine, Tongji University, 150 Jimo Road, Shanghai 200120, ChinaDepartment of Neurosurgery, Shanghai East Hospital, School of Medicine, Tongji University, 150 Jimo Road, Shanghai 200120, ChinaDepartment of Neurosurgery, Shanghai East Hospital, School of Medicine, Tongji University, 150 Jimo Road, Shanghai 200120, ChinaDepartment of Neurosurgery, Shanghai East Hospital, School of Medicine, Tongji University, 150 Jimo Road, Shanghai 200120, ChinaThe alpha-thalassemia mental retardation X-linked (ATRX) syndrome protein is a chromatin remodeling protein that primarily promotes the deposit of H3.3 histone variants in the telomere area. ATRX mutations not only cause ATRX syndrome but also influence development and promote cancer. The primary molecular characteristics of ATRX, including its molecular structures and normal and malignant biological roles, are reviewed in this article. We discuss the role of ATRX in its interactions with the histone variant H3.3, chromatin remodeling, DNA damage response, replication stress, and cancers, particularly gliomas, neuroblastomas, and pancreatic neuroendocrine tumors. ATRX is implicated in several important cellular processes and serves a crucial function in regulating gene expression and genomic integrity throughout embryogenesis. However, the nature of its involvement in the growth and development of cancer remains unknown. As mechanistic and molecular investigations on ATRX disclose its essential functions in cancer, customized therapies targeting ATRX will become accessible.https://www.mdpi.com/2072-6694/15/8/2228alpha-thalassemia mental retardation X-linked syndrome protein (ATRX)death-domain-associated protein (DAXX)DNA damagereplication stresstumorigenesis
spellingShingle Ying Pang
Xu Chen
Tongjie Ji
Meng Cheng
Rui Wang
Chunyu Zhang
Min Liu
Jing Zhang
Chunlong Zhong
The Chromatin Remodeler ATRX: Role and Mechanism in Biology and Cancer
Cancers
alpha-thalassemia mental retardation X-linked syndrome protein (ATRX)
death-domain-associated protein (DAXX)
DNA damage
replication stress
tumorigenesis
title The Chromatin Remodeler ATRX: Role and Mechanism in Biology and Cancer
title_full The Chromatin Remodeler ATRX: Role and Mechanism in Biology and Cancer
title_fullStr The Chromatin Remodeler ATRX: Role and Mechanism in Biology and Cancer
title_full_unstemmed The Chromatin Remodeler ATRX: Role and Mechanism in Biology and Cancer
title_short The Chromatin Remodeler ATRX: Role and Mechanism in Biology and Cancer
title_sort chromatin remodeler atrx role and mechanism in biology and cancer
topic alpha-thalassemia mental retardation X-linked syndrome protein (ATRX)
death-domain-associated protein (DAXX)
DNA damage
replication stress
tumorigenesis
url https://www.mdpi.com/2072-6694/15/8/2228
work_keys_str_mv AT yingpang thechromatinremodeleratrxroleandmechanisminbiologyandcancer
AT xuchen thechromatinremodeleratrxroleandmechanisminbiologyandcancer
AT tongjieji thechromatinremodeleratrxroleandmechanisminbiologyandcancer
AT mengcheng thechromatinremodeleratrxroleandmechanisminbiologyandcancer
AT ruiwang thechromatinremodeleratrxroleandmechanisminbiologyandcancer
AT chunyuzhang thechromatinremodeleratrxroleandmechanisminbiologyandcancer
AT minliu thechromatinremodeleratrxroleandmechanisminbiologyandcancer
AT jingzhang thechromatinremodeleratrxroleandmechanisminbiologyandcancer
AT chunlongzhong thechromatinremodeleratrxroleandmechanisminbiologyandcancer
AT yingpang chromatinremodeleratrxroleandmechanisminbiologyandcancer
AT xuchen chromatinremodeleratrxroleandmechanisminbiologyandcancer
AT tongjieji chromatinremodeleratrxroleandmechanisminbiologyandcancer
AT mengcheng chromatinremodeleratrxroleandmechanisminbiologyandcancer
AT ruiwang chromatinremodeleratrxroleandmechanisminbiologyandcancer
AT chunyuzhang chromatinremodeleratrxroleandmechanisminbiologyandcancer
AT minliu chromatinremodeleratrxroleandmechanisminbiologyandcancer
AT jingzhang chromatinremodeleratrxroleandmechanisminbiologyandcancer
AT chunlongzhong chromatinremodeleratrxroleandmechanisminbiologyandcancer