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Export of Misfolded Proteins out of the ER

Export of Misfolded Proteins out of the ER

After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...

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Updated: Jul 19, 2025

Measuring RAN Peptide Toxicity in C. elegans

Measuring RAN Peptide Toxicity in C. elegans

Published on: April 30, 2020

有毒物种影响ArfGAP-1功能

Simona Rossi^1,2, Michela Di Salvio³, Marilisa Balì⁴

¹Institute of Translational Pharmacology (IFT), National Research Council (CNR), 00133 Rome, Italy.

|August 11, 2023

概括

核细胞质运输中的缺陷,特别是来自C9orf72重复扩张的缺陷,扰乱了ALS中的RNA分布. 这项研究表明,有毒的C9orf72物种通过影响ArfGAP-1功能来损害膜贩运.

关键词:

在ArfGAP-1中使用.C9orf7272 是一个很好的方法.这种植物是Drosophila melanogaster.从戈尔吉到埃尔的非法贩运.骨髓缩侧面硬化症 (ALS) 是一种

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Last Updated: Jul 19, 2025

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科学领域:

神经科学是一个神经科学.
分子生物学分子生物学
细胞生物学细胞生物学

背景情况:

核细胞质运输对于细胞功能至关重要.
这种传输的缺陷与神经退行性疾病 (如ALS) 有关.
C9orf72重复扩张是ALS的主要遗传原因,并破坏了运输.

研究的目的:

为了识别由于C9orf72重复扩张而错位的特定mRNA.
调查C9orf72毒性对细胞传输通路的影响.
阐明C9orf72影响核细胞质分布的机制.

主要方法:

在人类和小鼠细胞中,G4C2六核酸重复的表达.
使用转录组分析系统识别改变的mRNA.
在培养细胞和Drosophila模型中进行功能研究.
研究ADP-化因子1 GTPase激活蛋白 (ArfGAP-1) 的功能.

主要成果:

在细胞核中观察到多元A mRNAs的积累.
参与膜贩运的mRNA在错位RNA中显著丰富.
C9orf72有毒物种,包括RNA重复和二重复蛋白 (C9-DPRs),损害了ArfGAP-1的功能.
逆向的戈尔吉-ER囊泡介导运输被确定为C9orf72毒性的目标.

结论:

C9orf72重复扩张破坏了核细胞质运输,导致mRNA错位.
由ArfGAP-1调节的膜贩运途径是ALS中C9orf72毒性的关键目标.
了解这种机制,可以了解ALS的发病过程和潜在的治疗策略.