Therapeutic vulnerabilities in the DNA damage response for the treatment of ATRX mutant neuroblastoma
Sign Up to like & getrecommendations! Published in 2020 at "EBioMedicine"
DOI: 10.1016/j.ebiom.2020.102971
Abstract: Background In neuroblastoma, genetic alterations in ATRX, define a distinct poor outcome patient subgroup. Despite the need for new therapies, there is a lack of available models and a dearth of pre-clinical research. Methods To… read more here.
Keywords: dna damage; atrx mutant; atrx; sensitivity ... See more keywords
ATRX immunohistochemistry can help refine ‘not elsewhere classified’ categorisation for grade II/III gliomas
Sign Up to like & getrecommendations! Published in 2019 at "British Journal of Neurosurgery"
DOI: 10.1080/02688697.2019.1600657
Abstract: Abstract Purpose: The 2016 WHO tumour classification highlights the role of IDH1/2 gene mutation and 1p/19q co-deletion in classifying grade II/III gliomas. A recent cIMPACT-NOW update proposes the use of the term ‘Not Elsewhere Classified’… read more here.
Keywords: immunohistochemistry; gliomas; atrx; 19q deletion ... See more keywords
X-linked α-thalassemia with mental retardation is downstream of protein kinase A in the meiotic cell cycle signaling cascade in Xenopus oocytes and is dynamically regulated in response to DNA damage†.
Sign Up to like & getrecommendations! Published in 2019 at "Biology of reproduction"
DOI: 10.1093/biolre/ioz001
Abstract: X-linked α-thalassemia with mental retardation (ATRX) is a chromatin remodeling protein that belongs to the SWItch/sucrose non-fermentable (SWI2/SNF2) family of helicase/ATPases. During meiosis, ATRX is necessary for heterochromatin formation and maintenance of chromosome stability in… read more here.
Keywords: protein; dna damage; response; dynamically regulated ... See more keywords
Functional crosstalk between the Fanconi anemia and ATRX/DAXX histone chaperone pathways promotes replication fork recovery.
Sign Up to like & getrecommendations! Published in 2019 at "Human molecular genetics"
DOI: 10.1093/hmg/ddz250
Abstract: Fanconi anemia (FA) is a chromosome instability syndrome characterized by increased cancer predisposition. Specifically, the FA pathway functions to protect genome stability during DNA replication. The central FA pathway protein, FANCD2, locates to stalled replication… read more here.
Keywords: replication fork; fanconi anemia; atrx; fork ... See more keywords
EPCO-21. ELUCIDATING THE FUNCTIONAL RELEVANCE OF ATRX-DEFICIENCY IN H3.3-G34-MUTANT DIFFUSE HEMISPHERIC GLIOMA
Sign Up to like & getrecommendations! Published in 2024 at "Neuro-Oncology"
DOI: 10.1093/neuonc/noae165.0020
Abstract: Diffuse hemispheric gliomas (DHGs) account for 30% of aggressive cerebral tumors in children and young adults, exhibiting abysmal outcomes. Co-existing recurrent mutations in H3.3 histones at residue G34 (H3.3-G34R/V mutation) and concurrent inactivating mutations in… read more here.
Keywords: atrx; atrx deficiency; g34 mutant; diffuse hemispheric ... See more keywords
PATH-105. GTAKA (Glioneuronal Tumor with ATRX Alteration, Kinase Fusion, and Anaplastic Features): A Novel Central Nervous System Entity with Distinct Clinicopathologic, Molecular Profiles, and Prognosis
Sign Up to like & getrecommendations! Published in 2025 at "Neuro-Oncology"
DOI: 10.1093/neuonc/noaf201.1057
Abstract: To investigate the clinicopathological, immunohistochemistry, molecular characteristics, and prognosis of new entity-GTAKA (Glioneuronal tumor with ATRX alteration, kinase fusion and anaplastic features). Seven GTAKA cases were collected and analyzed through histomorphological evaluation, immunohistochemistry (IHC), DNA… read more here.
Keywords: glioneuronal tumor; fusion; atrx; gtaka glioneuronal ... See more keywords
Abstract 3949: SMARCAL1 is a novel synthetic lethal target in ALT+ osteosarcoma and neuroblastoma
Sign Up to like & getrecommendations! Published in 2024 at "Cancer Research"
DOI: 10.1158/1538-7445.am2024-3949
Abstract: Osteosarcoma (OS) is an aggressive pediatric solid tumor that is difficult to treat with established therapies. We performed CRISPR/Cas9 screening in 13 OS cell lines as part of the Dependency Map project. Mining this data… read more here.
Keywords: dependency; smarcal1 novel; smarcal1; atrx ... See more keywords
ATRX regulates glial identity and the tumor microenvironment in IDH-mutant glioma
Sign Up to like & getrecommendations! Published in 2021 at "Genome Biology"
DOI: 10.1186/s13059-021-02535-4
Abstract: Background Recent single-cell transcriptomic studies report that IDH-mutant gliomas share a common hierarchy of cellular phenotypes, independent of genetic subtype. However, the genetic differences between IDH-mutant glioma subtypes are prognostic, predictive of response to chemotherapy,… read more here.
Keywords: mutant gliomas; idh mutant; glioma; atrx ... See more keywords
TOP3A amplification and ATRX inactivation are mutually exclusive events in pediatric osteosarcomas using ALT
Sign Up to like & getrecommendations! Published in 2022 at "EMBO Molecular Medicine"
DOI: 10.15252/emmm.202215859
Abstract: In some types of cancer, telomere length is maintained by the alternative lengthening of telomeres (ALT) mechanism. In many ALT cancers, the α‐thalassemia/mental retardation syndrome X‐linked (ATRX) gene is mutated leading to the conclusion that… read more here.
Keywords: atrx; pediatric osteosarcomas; alt; top3a amplification ... See more keywords
The Role of ATRX in Glioma Biology
Sign Up to like & getrecommendations! Published in 2017 at "Frontiers in Oncology"
DOI: 10.3389/fonc.2017.00236
Abstract: The current World Health Organization classification of CNS tumors has made a tremendous leap from past editions by incorporating molecular criteria in addition to the pre-existing histological parameters. The revised version has had a particular… read more here.
Keywords: atrx status; biology; role atrx; atrx ... See more keywords
The Chromatin Remodeler ATRX: Role and Mechanism in Biology and Cancer
Sign Up to like & getrecommendations! Published in 2023 at "Cancers"
DOI: 10.3390/cancers15082228
Abstract: Simple Summary ATRX is one of the most frequently mutated tumor suppressor genes in human cancers. ATRX protein is a chromatin remodeler and transcriptional regulator that is essential for normal development. ATRX plays a crucial… read more here.
Keywords: atrx; role; biology; chromatin remodeler ... See more keywords