数据日期 2026-10-29 · 生成时间 2026-10-29 06:03
研究问题
Cytosolic aggregates containing misfolded superoxide dismutase-1 (SOD1) protein is an established hallmark of ALS caused by mutations in the SOD1 gene.
方法
To test the hypothesis that CSF constitutes a route for the spread of SOD1-ALS, we performed intracister- nal injection of strain A and B SOD1 aggregates in adult asymptomatic hSOD1 Tg mice.；Materials & methods Mice In this study, we used transgenic hemizygous mice expressing G85R mutant human SOD1 (hSOD1G85R ) [7].；Acta Neuropathologica Communications (2026) 14:225 Antibodies The antibodies used in this study are in-house produced and cover almost the entire length of the 153 amino acid (aa) hSOD1 sequence.；To relieve pain, Carprofen (Rimadyl, 5 mg/kg, Pfizer) was injected subcutaneously.
核心结论
The mice dedicated to immunohistochemical analysis were perfused intra- cardially with saline followed by 10% buffered forma- lin and immersion fixed in the latter solution.；The homogenates were then diluted in 20 volumes of PBS containing 80 µl/ml Complete, 1.8 mM EDTA and 1 mM DTT and 1% NP40, sonicated for 30 s per sample and centrifuged at 1000 x g for 10 min at 4 °C.；After washing, the filter was blocked using 5% dry milk and 0.1% Tween for two hours. ‖ 结论：Our study shows that injection of hSOD1 seeds into CSF of adult asymptmatic hSODG85R Tg mice induced premature ALS-like disease and shortened survival sig- nificantly compared to control inoculated and non-inoc- ulated mice.
局限
With regard to the present design we could not dis- criminate between directional propagation or multifo- cal seeding from the CSF, which would be a question for future studies.
对本项目的价值
★★☆☆☆ 衰弱/肌少症是少有的已有监管认可终点的老年综合征，是临床转化的最短路径。最大样本量约 287；设计 Meta 分析、系统综述。
全文来源
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原文题录
- 英文标题：Intracisternal injection of two human SOD1 aggregate strains induces templated aggregation and ALS in transgenic mouse model.
- 期刊与标识：Acta Neuropathol Commun · PMID 42947273 · DOI 10.1186/s40478-026-02473-x**
A 级证据分级：A 级（同行评议人体研究）
本文属人体研究证据，但需注意：观察性或小样本干预的结论不等于已证实临床获益；效应量与长期安全性仍需更大规模、更长随访的试验验证。
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