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Article

FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis.

Acta neuropathologica communications - 25 Apr 2015

Kino Yoshihiro, Washizu Chika, Kurosawa Masaru, Yamada Mizuki, Miyazaki Haruko, Akagi Takumi, Hashikawa Tsutomu, Doi Hiroshi, Takumi Toru, Hicks Geoffrey G, Hattori Nobutaka, Shimogori Tomomi, Nukina Nobuyuki

Abstract excerpt

INTRODUCTION: FUS/TLS is an RNA-binding protein whose genetic mutations or pathological inclusions are associated with neurological diseases including amyotrophic lateral sclerosis (ALS), frontotemporal lobar degeneration, and essential tremor (ET). It is unclear whether their pathogenesis is mediated by gain or loss of function of FUS/TLS. RESULTS: Here, we established outbred FUS/TLS knockout mice to clarify...

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Source

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Identifiers

PMID: 25907258
DOI: 10.1186/s40478-015-0202-6
PMCID: PMC4408580

Genes

FUS

Provenance

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Related research

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FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis.PMID 25907258Read ↗

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ALS-linked cytoplasmic FUS assemblies are compositionally different from physiological stress granules and sequester hnRNPA3, a novel modifier of FUS toxicity.Semantic proximity Read ↗

ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP.Semantic proximity Read ↗

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