The function of RNA-binding proteins at the synapse: implications for neurodegeneration

CF Sephton, G Yu - Cellular and Molecular Life Sciences, 2015 - Springer
The loss of synapses is a central event in neurodegenerative diseases. Synaptic proteins
are often associated with disease neuropathology, but their role in synaptic loss is not fully …

De novo truncating FUS gene mutation as a cause of sporadic amyotrophic lateral sclerosis

M DeJesus‐Hernandez, J Kocerha, NC Finch… - Human …, 2010 - Wiley Online Library
Mutations in the gene encoding fused in sarcoma (FUS) were recently identified as a novel
cause of amyotrophic lateral sclerosis (ALS), emphasizing the genetic heterogeneity of ALS …

Fused in sarcoma: properties, self-assembly and correlation with neurodegenerative diseases

C Chen, X Ding, N Akram, S Xue, SZ Luo - Molecules, 2019 - mdpi.com
Fused in sarcoma (FUS) is a DNA/RNA binding protein that is involved in RNA metabolism
and DNA repair. Numerous reports have demonstrated by pathological and genetic analysis …

Role of FET proteins in neurodegenerative disorders

F Svetoni, P Frisone, MP Paronetto - RNA biology, 2016 - Taylor & Francis
Neurodegenerative disorders such as Alzheimer disease (AD), frontotemporal dementia
(FTD), amyotrophic lateral sclerosis (ALS), Parkinson disease (PD), Huntington's disease …

Intracellular localization and splicing regulation of FUS/TLS are variably affected by amyotrophic lateral sclerosis-linked mutations

Y Kino, C Washizu, E Aquilanti, M Okuno… - Nucleic acids …, 2011 - academic.oup.com
TLS (translocated in liposarcoma), also known as FUS (fused in sarcoma), is an RNA/DNA-
binding protein that plays regulatory roles in transcription, pre-mRNA splicing and mRNA …

Novel FUS/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosis

C Hewitt, J Kirby, JR Highley, JA Hartley… - Archives of …, 2010 - jamanetwork.com
Objective To determine the frequency of and clinicopathologic phenotypes associated
withFUS/TLSmutations in a large cohort of amyotrophic lateral sclerosis (ALS) cases from …

FUS regulates genes coding for RNA-binding proteins in neurons by binding to their highly conserved introns

T Nakaya, P Alexiou, M Maragkakis, A Chang… - Rna, 2013 - rnajournal.cshlp.org
Dominant mutations and mislocalization or aggregation of Fused in Sarcoma (FUS), an RNA-
binding protein (RBP), cause neuronal degeneration in Amyotrophic Lateral Sclerosis (ALS) …

Fused in sarcoma (FUS) in DNA repair: tango with poly (ADP-ribose) polymerase 1 and compartmentalisation of damaged DNA

MV Sukhanova, AS Singatulina, D Pastré… - International Journal of …, 2020 - mdpi.com
The fused in sarcoma (FUS) protein combines prion-like properties with a multifunctional
DNA/RNA-binding domain and has functions spanning the regulation of RNA metabolism …

FUS contributes to mTOR-dependent inhibition of translation

M Sévigny, IB Julien, JP Venkatasubramani… - Journal of Biological …, 2020 - ASBMB
The amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)–linked RNA-
binding protein called FUS (fused in sarcoma) has been implicated in several aspects of …

Protein quality control and the amyotrophic lateral sclerosis/frontotemporal dementia continuum

H Shahheydari, A Ragagnin, AK Walker… - Frontiers in molecular …, 2017 - frontiersin.org
Protein homeostasis, or proteostasis, has an important regulatory role in cellular function.
Protein quality control mechanisms, including protein folding and protein degradation …