SIRT2 inhibition as the Achilles’ heel of ATRX-deficient gliomas
ATRX is a chromatin remodeling protein that facilitates H3.3 deposition at telomeres and pericentromeric heterochromatin. It is essential for normal brain development, and mutations in ATRX cause alpha thalassemia X-linked intellectual disability (ATR-X) syndrome, a disorder characterized by severe...
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| Main Author: | |
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| Format: | Article (Journal) Editorial |
| Language: | English |
| Published: |
January 2024
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| In: |
Neuro-Oncology
Year: 2024, Volume: 26, Issue: 1, Pages: 68-69 |
| ISSN: | 1523-5866 |
| DOI: | 10.1093/neuonc/noad217 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1093/neuonc/noad217
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| Author Notes: | Şevin Turcan |
| Summary: | ATRX is a chromatin remodeling protein that facilitates H3.3 deposition at telomeres and pericentromeric heterochromatin. It is essential for normal brain development, and mutations in ATRX cause alpha thalassemia X-linked intellectual disability (ATR-X) syndrome, a disorder characterized by severe cognitive defects.1 In pediatric high-grade gliomas, recurrent loss-of-function mutations in ATRX are frequently reported with TP53 and H3F3A mutations.2 In adult gliomas, ATRX is mutated in astrocytomas, co-occurring with IDH1 and TP53 mutations, and in high-grade astrocytomas with piloid features, a recently described molecular class of IDH-wildtype glioma.3 Furthermore, ATRX-deficient tumors exhibit a distinct molecular landscape where telomere length is maintained in a telomerase-independent manner, known as alternative lengthening of telomeres (ALT).4 |
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| Item Description: | Veröffentlicht: 06 November 2023 Gesehen am 12.01.2024 |
| Physical Description: | Online Resource |
| ISSN: | 1523-5866 |
| DOI: | 10.1093/neuonc/noad217 |