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Updated: Feb 17, 2026

Measuring RAN Peptide Toxicity in C. elegans
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来自C9orf72的二聚重复蛋白聚PR破坏皮质神经元中的膜刺激性和突触功能

Shao-Ming Wang1, Jui-Cheng Chan2, Engkarat Supapatarnun3

  • 1Graduate Institute of Biomedical Sciences, College of Medicine, China Medical University, Taichung, Taiwan; Neuroscience and Brain Disease Center, China Medical University, Taichung, Taiwan.

Neurobiology of disease
|February 15, 2026
PubMed
概括

肌缩侧面硬化症 (ALS) 涉及C9orf72基因突变. 这项研究表明,神经元中的聚PR蛋白表达增加了兴奋能力,这种兴奋能力可以通过特定的药物逆转,提供潜在的ALS治疗策略.

关键词:
肌缩性侧面硬化症 (AMLS) 是一种疾病.C9orf7272 是一个很好的方法.过度兴奋性 过度兴奋性这是Poly-PR.

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Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining,...
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