The role of FUS gene variants in neurodegenerative diseases

H Deng, K Gao, J Jankovic - Nature Reviews Neurology, 2014 - nature.com
The neurodegenerative diseases are a diverse group of disorders characterized by
progressive loss of specific groups of neurons. These diseases affect different populations …

Genetic contribution of FUS to frontotemporal lobar degeneration

T Van Langenhove, J Van Der Zee, K Sleegers… - Neurology, 2010 - AAN Enterprises
Background: Recently, the FUS gene was identified as a new causal gene for amyotrophic
lateral sclerosis (ALS) in∼ 4% of patients with familial ALS. Since ALS and frontotemporal …

[HTML][HTML] ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects

H Qiu, S Lee, Y Shang, WY Wang… - The Journal of …, 2014 - Am Soc Clin Investig
Autosomal dominant mutations of the RNA/DNA binding protein FUS are linked to familial
amyotrophic lateral sclerosis (FALS); however, it is not clear how FUS mutations cause …

[HTML][HTML] Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age-and dose-dependent fashion

JC Mitchell, P McGoldrick, C Vance, T Hortobagyi… - Acta …, 2013 - Springer
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are
relentlessly progressive neurodegenerative disorders with overlapping clinical, genetic and …

Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss

J Scekic‐Zahirovic, O Sendscheid, H El Oussini… - The EMBO …, 2016 - embopress.org
FUS is an RNA‐binding protein involved in amyotrophic lateral sclerosis (ALS) and
frontotemporal dementia (FTD). Cytoplasmic FUS‐containing aggregates are often …

[HTML][HTML] Importance of functional loss of FUS in FTLD/ALS

S Ishigaki, G Sobue - Frontiers in molecular biosciences, 2018 - frontiersin.org
Fused in sarcoma (FUS) is an RNA binding protein that regulates RNA metabolism including
alternative splicing, transcription, and RNA transportation. FUS is genetically and …

[HTML][HTML] FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis

Y Kino, C Washizu, M Kurosawa, M Yamada… - Acta neuropathologica …, 2015 - Springer
Introduction FUS/TLS is an RNA-binding protein whose genetic mutations or pathological
inclusions are associated with neurological diseases including amyotrophic lateral sclerosis …

RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked …

JG Daigle, NA Lanson Jr, RB Smith… - Human molecular …, 2013 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) is an uncommon neurodegenerative disease caused by
degeneration of upper and lower motor neurons. Several genes, including SOD1, TDP-43 …

[HTML][HTML] Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis

C Verbeeck, Q Deng, M DeJesus-Hernandez… - Molecular …, 2012 - Springer
Background Mutations in the gene encoding the RNA-binding protein fused in sarcoma
(FUS) can cause familial and sporadic amyotrophic lateral sclerosis (ALS) and rarely …

[HTML][HTML] Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis

Y Shang, EJ Huang - Brain research, 2016 - Elsevier
Recent advances in the genetics of amyotrophic lateral sclerosis (ALS) have provided key
mechanistic insights to the pathogenesis of this devastating neurodegenerative disease …