Wild-type C9orf72 drives proteasomal dysfunction and mutant aggregates via a Stat1-Isg15 axis in Huntington's disease.

doi:10.1016/j.neurot.2026.e00970

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Article

Wild-type C9orf72 drives proteasomal dysfunction and mutant aggregates via a Stat1-Isg15 axis in Huntington's disease.

Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics - 1 Sept 2026

Chan Siew Chin, Tung Chih-Wei, Chang Chih-Yi, Su Ching-Chun, Chen Yi-Ching, Wu Po-Ming, Tung Chia-Yu, Chen Shih-Feng, Kuo Hsiao-Ying, Cheng Pei-Hsun, Chen Chuan-Mu, Yang Shang-Hsun

Abstract excerpt

Mutant C9orf72 has been extensively studied as a major genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia, and is also known to generate Huntington's disease (HD)-like phenocopies. However, despite this strong disease association, the role of wild-type C9orf72 (wt-C9orf72) in neurodegeneration remains largely unexplored. HD is a neurodegenerative disease, and characterized by the...

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Topics

Animals
STAT1 Transcription Factor
Huntington Disease
Ubiquitins
Humans

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Proteasome Endopeptidase Complex

Mice

Cytokines

C9orf72 Protein

Mice, Transgenic

Mutation

Signal Transduction

Proteotoxic Stress

Brain

Huntingtin Protein

Source

Open in PubMed Open full text

Identifiers

PMID: 42562736
DOI: 10.1016/j.neurot.2026.e00970
PMCID: PMC13615721

Genes

C9ORF72

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 23 Oct 2026.

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