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Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1

التفاصيل البيبلوغرافية
العنوان: Condensation properties of stress granules and processing bodies are compromised in myotonic dystrophy type 1
المؤلفون: Selma Gulyurtlu, Monika S. Magon, Patrick Guest, Panagiotis P. Papavasiliou, Kim D. Morrison, Alan R. Prescott, Judith E. Sleeman
المصدر: Disease Models & Mechanisms, Vol 15, Iss 7 (2022)
بيانات النشر: The Company of Biologists, 2022.
سنة النشر: 2022
المجموعة: LCC:Medicine
LCC:Pathology
مصطلحات موضوعية: llps, myotonic dystrophy type 1, p-bodies, stress granules, trinucleotide repeats, Medicine, Pathology, RB1-214
الوصف: RNA regulation in mammalian cells requires complex physical compartmentalisation, using structures thought to be formed by liquid-liquid phase separation. Disruption of these structures is implicated in numerous degenerative diseases. Myotonic dystrophy type 1 (DM1) is a multi-systemic trinucleotide repeat disorder resulting from an expansion of nucleotides CTG (CTGexp) in the DNA encoding DM1 protein kinase (DMPK). The cellular hallmark of DM1 is the formation of nuclear foci that contain expanded DMPK RNA (CUGexp) (with thymine instead of uracil). We report here the deregulation of stress granules (SGs) and processing bodies (P-bodies), two cytoplasmic structures key for mRNA regulation, in cell culture models of DM1. Alterations to the rates of formation and dispersal of SGs suggest an altered ability of cells to respond to stress associated with DM1, while changes to the structure and dynamics of SGs and P-bodies suggest that a widespread alteration to the biophysical properties of cellular structures is a consequence of the presence of CUGexp RNA.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 1754-8403
1754-8411
Relation: http://dmm.biologists.org/content/15/7/dmm049294; https://doaj.org/toc/1754-8403; https://doaj.org/toc/1754-8411
DOI: 10.1242/dmm.049294
URL الوصول: https://doaj.org/article/903258d2b7f94b81b35dc6b6d5b5fe78
رقم الأكسشن: edsdoj.903258d2b7f94b81b35dc6b6d5b5fe78
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:17548403
17548411
DOI:10.1242/dmm.049294