C9-ALS-Associated Proline-Arginine Dipeptide Repeat Protein Induces Activation of NLRP3 Inflammasome of HMC3 Microglia Cells by Binding of Complement Component 1 Q Subcomponent-Binding Protein (C1QBP), and Syringin Prevents This Effect
Amyotrophic lateral sclerosis (ALS) is a fatal disease in which motor neurons gradually degenerate. The mutation of the <i>C9orf72</i> gene is the main genetic cause of ALS (C9-ALS). One of its specific pathological features is the production of proline-arginine (PR) dipeptide repeat pro...
Main Authors: | Ru-Huei Fu, Chia-Wen Tsai, Shao-Chih Chiu, Shih-Ping Liu, Yu-Ting Chiang, Yun-Hua Kuo, Woei-Cherng Shyu, Shinn-Zong Lin |
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Format: | Article |
Language: | English |
Published: |
MDPI AG
2022-10-01
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Series: | Cells |
Subjects: | |
Online Access: | https://www.mdpi.com/2073-4409/11/19/3128 |
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