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GCN2 inhibition reduces mutant SOD1 clustering and toxicity and delays disease progression in an amyotrophic lateral sclerosis mouse model.

作者信息

Ortiz Didio Alberto, Peregrín Nuria, Valencia Miguel, Vinueza-Gavilanes Rodrigo, Marín-Ordovas Elisa, Ferrero Roberto, Nicolás María Jesús, González-Aseguinolaza Gloria, Arrasate Montserrat, Aragón Tomás

机构信息

DNA and RNA Medicine Program, Center for Applied Medical Research (CIMA), University of Navarra, Pamplona, Spain.

Biomedical Engineering Program, Center for Applied Medical Research (CIMA), University of Navarra, Pamplona, Spain.

出版信息

Transl Neurodegener. 2024 Sep 20;13(1):49. doi: 10.1186/s40035-024-00441-w.

DOI:10.1186/s40035-024-00441-w
PMID:39304897
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11414287/
Abstract
摘要
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d5a/11414287/719e175e39c4/40035_2024_441_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d5a/11414287/719e175e39c4/40035_2024_441_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d5a/11414287/719e175e39c4/40035_2024_441_Fig1_HTML.jpg

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EMBO Rep. 2024 May;25(5):2479-2510. doi: 10.1038/s44319-024-00140-7. Epub 2024 Apr 29.
2
Molecular Mechanisms of Phase Separation and Amyloidosis of ALS/FTD-linked FUS and TDP-43.ALS/FTD 相关 FUS 和 TDP-43 的相分离和淀粉样变性的分子机制。
Aging Dis. 2024 Oct 1;15(5):2084-2112. doi: 10.14336/AD.2023.1118.
3
The Role and Therapeutic Potential of the Integrated Stress Response in Amyotrophic Lateral Sclerosis.
Molecules. 2025 Feb 5;30(3):694. doi: 10.3390/molecules30030694.
整合应激反应在肌萎缩侧索硬化症中的作用和治疗潜力。
Int J Mol Sci. 2022 Jul 15;23(14):7823. doi: 10.3390/ijms23147823.
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Fine tuning of the unfolded protein response by ISRIB improves neuronal survival in a model of amyotrophic lateral sclerosis.通过 ISRIB 对未折叠蛋白反应的微调可改善肌萎缩侧索硬化症模型中的神经元存活。
Cell Death Dis. 2020 May 26;11(5):397. doi: 10.1038/s41419-020-2601-2.
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The integrated stress response: From mechanism to disease.整体应激反应：从机制到疾病。
Science. 2020 Apr 24;368(6489). doi: 10.1126/science.aat5314.
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Repeat-associated non-AUG (RAN) translation mechanisms are running into focus for GGGGCC-repeat associated ALS/FTD.重复相关非 AUG（RAN）翻译机制成为 GGGGCC 重复相关肌萎缩侧索硬化症/额颞叶痴呆的研究焦点。
Prog Neurobiol. 2019 Dec;183:101697. doi: 10.1016/j.pneurobio.2019.101697. Epub 2019 Sep 21.
7
The UPR-PERK pathway is not a promising therapeutic target for mutant SOD1-induced ALS.UPR-PERK 通路不是突变 SOD1 诱导的 ALS 的有前途的治疗靶点。
Neurobiol Dis. 2019 Jul;127:527-544. doi: 10.1016/j.nbd.2019.03.024. Epub 2019 Mar 26.
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