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...
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MDPI AG
2023-04-01
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Series: | Cancers |
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Online Access: | https://www.mdpi.com/2072-6694/15/8/2228 |
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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 |
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