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000276275 1001_ $$aMoens, Thomas G$$b0
000276275 245__ $$aAmyotrophic lateral sclerosis caused by FUS mutations: advances with broad implications.
000276275 260__ $$aLondon$$bLancet Publ. Group$$c2025
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000276275 520__ $$aAutosomal dominant mutations in the gene encoding the DNA and RNA binding protein FUS are a cause of amyotrophic lateral sclerosis (ALS), and about 0·3-0·9% of patients with ALS are FUS mutation carriers. FUS-mutation-associated ALS (FUS-ALS) is characterised by early onset and rapid progression, compared with other forms of ALS. However, different pathogenic mutations in FUS can result in markedly different age at symptom onset and rate of disease progression. Most FUS mutations disrupt its nuclear localisation, leading to its cytoplasmic accumulation in the CNS. FUS also forms inclusions in around 5% of patients with the related neurodegenerative condition frontotemporal dementia. However, there are key differences between the two diseases at the genetic and neuropathological level, which suggest distinct pathogenic processes. Experimental models have uncovered potential pathogenic mechanisms in FUS-ALS and informed therapeutic strategies that are currently in development, including the silencing of FUS expression using an intrathecally administered antisense oligonucleotide.
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000276275 650_7 $$2NLM Chemicals$$aRNA-Binding Protein FUS
000276275 650_7 $$2NLM Chemicals$$aFUS protein, human
000276275 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: genetics
000276275 650_2 $$2MeSH$$aHumans
000276275 650_2 $$2MeSH$$aRNA-Binding Protein FUS: genetics
000276275 650_2 $$2MeSH$$aMutation: genetics
000276275 650_2 $$2MeSH$$aAnimals
000276275 7001_ $$aDa Cruz, Sandrine$$b1
000276275 7001_ $$0P:(DE-2719)2810592$$aNeumann, Manuela$$b2$$udzne
000276275 7001_ $$aShelkovnikova, Tatyana A$$b3
000276275 7001_ $$aShneider, Neil A$$b4
000276275 7001_ $$aVan Den Bosch, Ludo$$b5
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