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 …

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

S Sun, SC Ling, J Qiu, CP Albuquerque, Y Zhou… - Nature …, 2015 - nature.com
The RNA-binding protein FUS/TLS, mutation in which is causative of the fatal motor neuron
disease amyotrophic lateral sclerosis (ALS), is demonstrated to directly bind to the U1 …

TDP-43 and FUS RNA-binding proteins bind distinct sets of cytoplasmic messenger RNAs and differently regulate their post-transcriptional fate in motoneuron-like …

C Colombrita, E Onesto, F Megiorni, A Pizzuti… - Journal of Biological …, 2012 - ASBMB
The RNA-binding proteins TDP-43 and FUS form abnormal cytoplasmic aggregates in
affected tissues of patients with amyotrophic lateral sclerosis and frontotemporal lobar …

Dysregulated molecular pathways in amyotrophic lateral sclerosis–frontotemporal dementia spectrum disorder

FB Gao, S Almeida, R Lopez‐Gonzalez - The EMBO journal, 2017 - embopress.org
Frontotemporal dementia (FTD), the second most common form of dementia in people under
65 years of age, is characterized by progressive atrophy of the frontal and/or temporal lobes …

ALS/FTD‐associated FUS activates GSK‐3β to disrupt the VAPB–PTPIP 51 interaction and ER–mitochondria associations

R Stoica, S Paillusson, P Gomez‐Suaga, JC Mitchell… - EMBO …, 2016 - embopress.org
Defective FUS metabolism is strongly associated with amyotrophic lateral sclerosis and
frontotemporal dementia (ALS/FTD), but the mechanisms linking FUS to disease are not …

Local RNA translation at the synapse and in disease

L Liu-Yesucevitz, GJ Bassell, AD Gitler… - Journal of …, 2011 - Soc Neuroscience
Local regulation of protein synthesis in neurons has emerged as a leading research focus
because of its importance in synaptic plasticity and neurological diseases. The complexity of …

ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules

C Vance, EL Scotter, AL Nishimura… - Human molecular …, 2013 - academic.oup.com
Mutations in the gene encoding Fused in Sarcoma (FUS) cause amyotrophic lateral
sclerosis (ALS), a fatal neurodegenerative disorder. FUS is a predominantly nuclear DNA …

Genetics of amyotrophic lateral sclerosis: an update

S Chen, P Sayana, X Zhang, W Le - Molecular neurodegeneration, 2013 - Springer
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder involving
both upper motor neurons (UMN) and lower motor neurons (LMN). Enormous research has …

[PDF][PDF] Loss of dynamic RNA interaction and aberrant phase separation induced by two distinct types of ALS/FTD-linked FUS mutations

AG Niaki, J Sarkar, X Cai, K Rhine, V Vidaurre, B Guy… - Molecular cell, 2020 - cell.com
FUS is a nuclear RNA-binding protein, and its cytoplasmic aggregation is a pathogenic
signature of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). It …

Dysregulated mi RNA biogenesis downstream of cellular stress and ALS‐causing mutations: a new mechanism for ALS

A Emde, C Eitan, LL Liou, RT Libby, N Rivkin… - The EMBO …, 2015 - embopress.org
Interest in RNA dysfunction in amyotrophic lateral sclerosis (ALS) recently aroused upon
discovering causative mutations in RNA‐binding protein genes. Here, we show that …