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

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...

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Bibliographic Details
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
Format: Article
Language:English
Published: MDPI AG 2022-10-01
Series:Cells
Subjects:
amyotrophic lateral sclerosis (ALS)
<i>C9orf72</i>
proline-arginine dipeptide repeat protein (PR-DPR)
HMC3 microglia cell
NLRP3 inflammasome
mouse motor neuron NSC34 cell
Online Access:https://www.mdpi.com/2073-4409/11/19/3128

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https://www.mdpi.com/2073-4409/11/19/3128

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