线粒体功能是核糖体相关疾病的核心吗?

IF 18.1 1区 生物学 Q1 CELL BIOLOGY
Trends in Cell Biology Pub Date : 2025-10-01 Epub Date: 2025-08-26 DOI:10.1016/j.tcb.2025.07.007
Qiuxia Zhao, Elif Sarinay Cenik
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引用次数: 0

摘要

核糖体机制缺陷导致核糖体疾病,如钻石黑扇贫血,通常与蛋白质合成受损有关。然而,新出现的证据表明,线粒体功能障碍是核糖体功能不全的关键下游后果。因此,受损的能量代谢,而不是单独的翻译,是核糖体疾病的关键驱动因素,如钻石黑扇贫血?这一见解可以重新构建我们对疾病机制的理解,并可以确定代谢途径作为有希望的治疗靶点。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Is mitochondrial function at the heart of ribosome-related diseases?

Defects in ribosomal machinery cause ribosomopathies such as Diamond Blackfan anemia, classically linked to impaired protein synthesis. However, emerging evidence places mitochondrial dysfunction as a critical downstream consequence of ribosomal insufficiency. Thus, is impaired energy metabolism, rather than translation alone, a key driver of ribosomopathies such as Diamond Blackfan anemia? This insight could reframe our understanding of disease mechanisms and could identify metabolic pathways as promising therapeutic targets.

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来源期刊
Trends in Cell Biology
Trends in Cell Biology 生物-细胞生物学
CiteScore
32.00
自引率
0.50%
发文量
160
审稿时长
61 days
期刊介绍: Trends in Cell Biology stands as a prominent review journal in molecular and cell biology. Monthly review articles track the current breadth and depth of research in cell biology, reporting on emerging developments and integrating various methods, disciplines, and principles. Beyond Reviews, the journal features Opinion articles that follow trends, offer innovative ideas, and provide insights into the implications of new developments, suggesting future directions. All articles are commissioned from leading scientists and undergo rigorous peer-review to ensure balance and accuracy.
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