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Defects in translation-dependent quality control pathways lead to convergent molecular and neurodevelopmental pathology

التفاصيل البيبلوغرافية
العنوان: Defects in translation-dependent quality control pathways lead to convergent molecular and neurodevelopmental pathology
المؤلفون: Markus Terrey, Scott I Adamson, Jeffrey H Chuang, Susan L Ackerman
المصدر: eLife, Vol 10 (2021)
بيانات النشر: eLife Sciences Publications Ltd, 2021.
سنة النشر: 2021
المجموعة: LCC:Medicine
LCC:Science
LCC:Biology (General)
مصطلحات موضوعية: cerebellum, neurogenesis, translation, ribosome, n-Tr20, Medicine, Science, Biology (General), QH301-705.5
الوصف: Translation-dependent quality control pathways such as no-go decay (NGD), non-stop decay (NSD), and nonsense-mediated decay (NMD) govern protein synthesis and proteostasis by resolving non-translating ribosomes and preventing the production of potentially toxic peptides derived from faulty and aberrant mRNAs. However, how translation is altered and the in vivo defects that arise in the absence of these pathways are poorly understood. Here, we show that the NGD/NSD factors Pelo and Hbs1l are critical in mice for cerebellar neurogenesis but expendable for survival of these neurons after development. Analysis of mutant mouse embryonic fibroblasts revealed translational pauses, alteration of signaling pathways, and translational reprogramming. Similar effects on signaling pathways, including mTOR activation, the translatome and mouse cerebellar development were observed upon deletion of the NMD factor Upf2. Our data reveal that these quality control pathways that function to mitigate errors at distinct steps in translation can evoke similar cellular responses.
نوع الوثيقة: article
وصف الملف: electronic resource
اللغة: English
تدمد: 2050-084X
Relation: https://elifesciences.org/articles/66904; https://doaj.org/toc/2050-084X
DOI: 10.7554/eLife.66904
URL الوصول: https://doaj.org/article/d433693e93854cab9317df076693e5c5
رقم الأكسشن: edsdoj.433693e93854cab9317df076693e5c5
قاعدة البيانات: Directory of Open Access Journals
الوصف
تدمد:2050084X
DOI:10.7554/eLife.66904