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Amyotrophic lateral sclerosis/Motor neuron disease (ALS/MND) is an adult onset motoneuron degenerative disease with a lifetime risk of ~1/1000. Approximately 80% of the cases are fatal within five years of diagnosis. There is no cure and only one FDA approved therapy, Riluzole has a minor effect on the progression of the disease.
Mutations in genes such as SOD1, TDP-43, FUS, ANG, and VAPB also cause some forms of ALS although most forms of ALS is sporadic in nature. Despite the identification many genes causing ALS the exact mechanism of motor neuron toxicity is unclear, although a variety of mechanisms have been postulated.
Identifying the upstream events that result in toxicity is critical to impact the disease process. Protein misfolding and cellular inclusions are a common theme in many neurodegenerative diseases including ALS. How protein misfolding contributes to toxicity, if protein inclusions are protective or detrimental is presently unknown.
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论文共 40 篇作者统计合作学者相似作者
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David J Burrows,Alexander McGown, Olfat Abduljabbar,Lydia M Castelli, Pamela J Shaw,Guillaume M Hautbergue,Tennore M Ramesh
biorxiv(2024)
Liam Kempthorne, Deniz Vaizoglu, Alexander J Cammack, Mireia Carcole, Pacharaporn Suklai,Bhavana Muralidharan,Francois Kroll,Thomas G Moens,Lidia Yshii,Stijn Verschoren, Benedikt V Holbling, Eszter Katona,
biorxiv(2024)
Johnathan Cooper-Knock,Tobias Moll,Tennore Ramesh,Lydia Castelli, Alexander Beer, Henry Robins,Ian Fox,Isabell Niedermoser,Philip Van Damme,Matthieu Moisse,Wim Robberecht,Orla Hardiman,
semanticscholar(2019)
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David John Burrows,Alexander McGown, Saurabh A Jain,Milena De Felice,Tennore M Ramesh,Basil Sharrack,Arshad Majid
Johnathan Cooper-Knock,Tobias Moll,Tennore Ramesh,Lydia Castelli, Alexander Beer, Henry Robins,Ian Fox, Isabell Niedermoser,Philip Van Damme,Matthieu Moisse,Wim Robberecht,Orla Hardiman,
Cell Reportsno. 9 (2019): 2298-2306.e5
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