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 …

Superoxide dismutase 1 in health and disease: how a frontline antioxidant becomes neurotoxic

BG Trist, JB Hilton, DJ Hare, PJ Crouch… - Angewandte Chemie …, 2021 - Wiley Online Library
Cu/Zn superoxide dismutase (SOD1) is a frontline antioxidant enzyme catalysing superoxide
breakdown and is important for most forms of eukaryotic life. The evolution of aerobic …

Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway

YC Lin, MS Kumar, N Ramesh, EN Anderson… - Nature …, 2021 - nature.com
Nucleocytoplasmic transport (NCT) decline occurs with aging and neurodegeneration. Here,
we investigated the NCT pathway in models of amyotrophic lateral sclerosis–fused in …

Single copy/knock-in models of ALS SOD1 in C. elegans suggest loss and gain of function have different contributions to cholinergic and glutamatergic …

SN Baskoylu, J Yersak, P O'Hern, S Grosser… - PLoS …, 2018 - journals.plos.org
Mutations in Cu/Zn superoxide dismutase 1 (SOD1) lead to Amyotrophic Lateral Sclerosis
(ALS), a neurodegenerative disease that disproportionately affects glutamatergic and …

Nuclear export inhibition enhances HLH-30/TFEB activity, autophagy, and lifespan

MJ Silvestrini, JR Johnson, AV Kumar, TG Thakurta… - Cell reports, 2018 - cell.com
Transcriptional modulation of the process of autophagy involves the transcription factor HLH-
30/TFEB. In order to systematically determine the regulatory network of HLH-30/TFEB, we …

Pathological insights from amyotrophic lateral sclerosis animal models: comparisons, limitations, and challenges

L Zhu, S Li, XJ Li, P Yin - Translational neurodegeneration, 2023 - Springer
In order to dissect amyotrophic lateral sclerosis (ALS), a multigenic, multifactorial, and
progressive neurodegenerative disease with heterogeneous clinical presentations …

[HTML][HTML] In vitro and in vivo models of amyotrophic lateral sclerosis: an updated overview

AM Gois, DMF Mendonça, MAM Freire… - Brain Research Bulletin, 2020 - Elsevier
Abstract Amyotrophic Lateral Sclerosis (ALS) is a progressive, neurodegenerative disease
characterized by loss of upper motor neurons (UMN) and lower motor neurons (LMN) …

Amyotrophic lateral sclerosis: proteins, proteostasis, prions, and promises

L McAlary, YL Chew, JS Lum, NJ Geraghty… - Frontiers in Cellular …, 2020 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is characterized by the progressive degeneration of the
motor neurons that innervate muscle, resulting in gradual paralysis and culminating in the …

Drosophila melanogaster as a model to study autophagy in neurodegenerative diseases induced by proteinopathies

S Santarelli, C Londero, A Soldano… - Frontiers in …, 2023 - frontiersin.org
Proteinopathies are a large group of neurodegenerative diseases caused by both genetic
and sporadic mutations in particular genes which can lead to alterations of the protein …

Genetic strategies to tackle neurological diseases in fruit flies

M Şentürk, HJ Bellen - Current Opinion in Neurobiology, 2018 - Elsevier
Highlights•Expression of reference and variant human genes in flies permit functional
testing.•Innovative genetic strategies in flies drive discovery of disease associated …