ALS genetics: gains, losses, and implications for future therapies

G Kim, O Gautier, E Tassoni-Tsuchida, XR Ma… - Neuron, 2020 - cell.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder caused by the loss
of motor neurons from the brain and spinal cord. The ALS community has made remarkable …

Protein aggregation in amyotrophic lateral sclerosis

AM Blokhuis, EJN Groen, M Koppers… - Acta …, 2013 - Springer
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have …

Drosophila as an In Vivo Model for Human Neurodegenerative Disease

L McGurk, A Berson, NM Bonini - Genetics, 2015 - academic.oup.com
With the increase in the ageing population, neurodegenerative disease is devastating to
families and poses a huge burden on society. The brain and spinal cord are extraordinarily …

Neuromuscular junction dysfunction in amyotrophic lateral sclerosis

S Verma, S Khurana, A Vats, B Sahu, NK Ganguly… - Molecular …, 2022 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …

[HTML][HTML] 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 …

Nearly 30 years of animal models to study amyotrophic lateral sclerosis: a historical overview and future perspectives

T Bonifacino, RA Zerbo, M Balbi, C Torazza… - International journal of …, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal, multigenic, multifactorial, and non-cell
autonomous neurodegenerative disease characterized by upper and lower motor neuron …

Mechanisms, models and biomarkers in amyotrophic lateral sclerosis

MR Turner, R Bowser, L Bruijn, L Dupuis… - … Lateral Sclerosis and …, 2013 - Taylor & Francis
The last 30 years have seen a major advance in the understanding of the clinical and
pathological heterogeneity of amyotrophic lateral sclerosis (ALS), and its overlap with …

Small molecules for modulating protein driven liquid-liquid phase separation in treating neurodegenerative disease

RJ Wheeler, HO Lee, I Poser, A Pal, T Doeleman… - 2019 - europepmc.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with few avenues for
treatment. Many proteins implicated in ALS associate with stress granules, which are …

Fused in Sarcoma (FUS) Protein Lacking Nuclear Localization Signal (NLS) and Major RNA Binding Motifs Triggers Proteinopathy and Severe Motor Phenotype in …

TA Shelkovnikova, OM Peters, AV Deykin… - Journal of biological …, 2013 - ASBMB
Dysfunction of two structurally and functionally related proteins, FUS and TAR DNA-binding
protein of 43 kDa (TDP-43), implicated in crucial steps of cellular RNA metabolism can …

Neuromuscular junction dismantling in amyotrophic lateral sclerosis

V Cappello, M Francolini - International journal of molecular sciences, 2017 - mdpi.com
Neuromuscular junction assembly and plasticity during embryonic, postnatal, and adult life
are tightly regulated by the continuous cross-talk among motor nerve endings, muscle fibers …