Glycine-Alanine Dipeptide Repeat Protein from C9-ALS Interacts with Sulfide Quinone Oxidoreductase (SQOR) to Induce the Activity of the NLRP3 Inflammasome in HMC3 Microglia: Irisflorentin Reverses This Interaction

Glycine-Alanine Dipeptide Repeat Protein from C9-ALS Interacts with Sulfide Quinone Oxidoreductase (SQOR) to Induce the Activity of the NLRP3 Inflammasome in HMC3 Microglia: Irisflorentin Reverses This Interaction

Amyotrophic lateral sclerosis (ALS) is a fatal rare disease of progressive degeneration of motor neurons. The most common genetic mutation in ALS is the hexanucleotide repeat expansion (HRE) located in the first intron of the C9orf72 gene (C9-ALS). HRE can produce dipeptide repeat proteins (DPRs) su...

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Bibliographic Details
Main Authors: Ru-Huei Fu, Hui-Jye Chen, Syuan-Yu Hong
Format: Article
Language:English
Published: MDPI AG 2023-10-01
Series:Antioxidants
Subjects:
amyotrophic lateral sclerosis (ALS)
C9ORF72
glycine-alanine dipeptide repeat proteins (GA-DPRs)
NLRP3 inflammasome
sulfide quinone oxidoreductase (SQOR)
ROS
Online Access:https://www.mdpi.com/2076-3921/12/10/1896

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