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 …

Where and why modeling amyotrophic lateral sclerosis

F Liguori, S Amadio, C Volonté - International Journal of Molecular …, 2021 - mdpi.com
Over the years, researchers have leveraged a host of different in vivo models in order to
dissect amyotrophic lateral sclerosis (ALS), a neurodegenerative/neuroinflammatory …

Investigating the sequence determinants of the curling of amyloid fibrils using ovalbumin as a case study

JAJ Housmans, B Houben, M Monge-Morera… - …, 2022 - ACS Publications
Highly ordered, straight amyloid fibrils readily lend themselves to structure determination
techniques and have therefore been extensively characterized. However, the less ordered …

Artepillin C, a major component of Brazilian green propolis, inhibits endoplasmic reticulum stress and protein aggregation

Y Hirata, M Motoyama, S Kimura, M Takashima… - European journal of …, 2021 - Elsevier
Propolis, a compound produced by honeybees, has long been used in food and beverages
to improve health and prevent diseases. We previously reported that the ethanol extracts of …

Molecular Mechanisms of Aggregation of Canine SOD1 E40K Amyloidogenic Mutant Protein

K Wakayama, S Kimura, Y Kobatake, H Kamishina… - Molecules, 2022 - mdpi.com
Canine degenerative myelopathy (DM) is a human amyotrophic lateral sclerosis (ALS)-like
neurodegenerative disease. It is a unique, naturally occurring animal model of human ALS …

Prevalence of SOD1 allele associated with degenerative myelopathy in canine population in Greece

A Kountourantzis, S Minoudi, N Karaiskou… - Research in Veterinary …, 2023 - Elsevier
Canine degenerative myelopathy (CDM) is a late-onset fatal disorder associated with a point
mutation of the superoxide dismutase 1 (SOD1) gene (c. 118G> A). The purpose of this …

The usage and advantages of several common amyotrophic lateral sclerosis animal models

L Zhou, M Xie, X Wang, R Xu - Frontiers in Neuroscience, 2024 - frontiersin.org
Amyotrophic lateral sclerosis is a fatal, multigenic, multifactorial neurodegenerative disease
characterized by upper and lower motor neuron loss. Animal models are essential for …

The inhibitory effects of MIF on accumulation of canine degenerative myelopathy-associated mutant SOD1 aggregation

S Nomura, Y Kobatake, S Takashima… - Research in Veterinary …, 2022 - Elsevier
Canine degenerative myelopathy (DM) is a progressive neurodegenerative disorder, which
is commonly associated with c. 118G> A (p. E40K) missense mutation in the superoxide …

Novel oxindole compounds inhibit the aggregation of amyloidogenic proteins associated with neurodegenerative diseases

S Kimura, H Kamishina, Y Hirata, K Furuta… - … et Biophysica Acta (BBA …, 2022 - Elsevier
Amyloidogenic proteins form aggregates in cells, thereby leading to neurodegenerative
disorders, including Alzheimer's and prion's disease, amyotrophic lateral sclerosis (ALS) in …

Intrinsic structural vulnerability in the hydrophobic core induces species-specific aggregation of canine SOD1 with degenerative myelopathy–linked E40K mutation

K Hashimoto, S Watanabe, M Akutsu, N Muraki… - Journal of Biological …, 2023 - ASBMB
Canine degenerative myelopathy (DM), a fatal neurodegenerative disease in dogs, shares
clinical and genetic features with amyotrophic lateral sclerosis, a human motor neuron …