Lost in local translation: TDP-43 and FUS in axonal/neuromuscular junction maintenance and dysregulation in amyotrophic lateral sclerosis

D Piol, T Robberechts, S Da Cruz - Neuron, 2023 - cell.com
Key early features of amyotrophic lateral sclerosis (ALS) are denervation of neuromuscular
junctions and axonal degeneration. Motor neuron homeostasis relies on local translation …

Disruption of MAM integrity in mutant FUS oligodendroglial progenitors from hiPSCs

Y Zhu, T Burg, K Neyrinck, T Vervliet, F Nami… - Acta …, 2024 - Springer
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and fatal neurodegenerative
disorder, characterized by selective loss of motor neurons (MNs). A number of causative …

Cytoplasmic aggregation of mutant FUS causes multistep RNA splicing perturbations in the course of motor neuron pathology

AP Rezvykh, AA Ustyugov, KD Chaprov… - Nucleic acids …, 2023 - academic.oup.com
Dysfunction of the RNA-binding protein (RBP) FUS implicated in RNA metabolism can
cause amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. Mutations …

Amyotrophic lateral sclerosis caused by FUS mutations: advances with broad implications

TG Moens, S Da Cruz, M Neumann… - The Lancet …, 2025 - thelancet.com
Autosomal 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 …

Engineered NLS-chimera downregulates expression of aggregation-prone endogenous FUS

M Hayashi, A Girdhar, YH Ko, KM Kim… - Nature …, 2024 - nature.com
Importin β-superfamily nuclear import receptors (NIRs) mitigate mislocalization and
aggregation of RNA-binding proteins (RBPs), like FUS and TDP-43, which are implicated in …

Mutant FUS induces chromatin reorganization in the hippocampus and alters memory processes

L Tzeplaeff, J Seguin, S Le Gras, S Megat… - Progress in …, 2023 - Elsevier
Cytoplasmic mislocalization of the nuclear Fused in Sarcoma (FUS) protein is associated to
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Cytoplasmic FUS …

[HTML][HTML] FUS-mediated HypEVs: neuroprotective effects against ischemic stroke

Y Wu, X Huang, Z Tan, J Zang, M Peng, N He… - Bioactive Materials, 2023 - Elsevier
Few studies have investigated the properties and protein composition of small extracellular
vesicles (sEVs) derived from neurons under hypoxic conditions. Presently, the extent of the …

Functional consequences of familial ALS‐associated SOD1L84F in neuronal and muscle cells

S Verma, A Vats, V Ahuja, K Vats, S Khurana… - The FASEB …, 2024 - Wiley Online Library
Amyotrophic lateral sclerosis is a fatal neurodegenerative disorder characterized by
progressive skeletal muscle denervation and loss of motor neurons that results in muscle …

Animal models of FUS-proteinopathy: a systematic review

A Rezvykh, D Shteinberg, E Bronovitsky… - Biochemistry …, 2024 - Springer
Mutations that disrupt the function of the DNA/RNA-binding protein FUS could cause
amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases. One of the key …

A scalable screening platform for phenotypic subty** of ALS patient-derived fibroblasts

K Kumbier, M Roth, Z Li, J Lazzari-Dean, C Waters… - bioRxiv, 2022 - biorxiv.org
ABSTRACT A major challenge for understanding and treating Amyotrophic Lateral Sclerosis
(ALS) is that most patients have no known genetic cause. Even within defined genetic …