SIRT2 inhibition as the Achilles’ heel of ATRX-deficient gliomas

• Verfasst von: Turcan, S̨evin [VerfasserIn]
• Titel: SIRT2 inhibition as the Achilles’ heel of ATRX-deficient gliomas
• Verf.angabe: Şevin Turcan
• E-Jahr: 2024
• Jahr: January 2024
• Umfang: 2 S.
• Fussnoten: Veröffentlicht: 06 November 2023 ; Gesehen am 12.01.2024
• Titel Quelle: Enthalten in: Neuro-Oncology
• Ort Quelle: Oxford : Oxford Univ. Press, 1999
• Jahr Quelle: 2024
• Band/Heft Quelle: 26(2024), 1, Seite 68-69
• ISSN Quelle: 1523-5866
• DOI: doi:10.1093/neuonc/noad217
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 187796302X

Abstract

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