Animal models of neurodegenerative diseases

TM Dawson, TE Golde, C Lagier-Tourenne - Nature neuroscience, 2018 - nature.com
Animal models of adult-onset neurodegenerative diseases have enhanced the
understanding of the molecular pathogenesis of Alzheimer's disease, Parkinson's disease …

[HTML][HTML] Genetics of amyotrophic lateral sclerosis: seeking therapeutic targets in the era of gene therapy

N Suzuki, A Nishiyama, H Warita, M Aoki - Journal of human genetics, 2023 - nature.com
Amyotrophic lateral sclerosis (ALS) is an intractable disease that causes respiratory failure
leading to mortality. The main locus of ALS is motor neurons. The success of antisense …

[HTML][HTML] Human sensorimotor organoids derived from healthy and amyotrophic lateral sclerosis stem cells form neuromuscular junctions

JD Pereira, DM DuBreuil, AC Devlin, A Held… - Nature …, 2021 - nature.com
Human induced pluripotent stem cells (iPSC) hold promise for modeling diseases in
individual human genetic backgrounds and thus for developing precision medicine. Here …

[HTML][HTML] Molecular and cellular mechanisms affected in ALS

L Le Gall, E Anakor, O Connolly… - Journal of personalized …, 2020 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a terminal late-onset condition characterized by the
loss of upper and lower motor neurons. Mutations in more than 30 genes are associated to …

[HTML][HTML] 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 …

[HTML][HTML] Are astrocytes the predominant cell type for activation of Nrf2 in aging and neurodegeneration?

JR Liddell - Antioxidants, 2017 - mdpi.com
Nuclear factor erythroid 2-related factor 2 (Nrf2) is a transcription factor that regulates
hundreds of antioxidant genes, and is activated in response to oxidative stress. Given that …

Mouse models of ALS: Past, present and future

C Lutz - Brain Research, 2018 - Elsevier
Genome sequencing of both sporadic and familial patients of Amyotrophic Lateral Sclerosis
(ALS) has led to the identification of new genes that are both contributing and causative in …

[HTML][HTML] The neglected genes of ALS: cytoskeletal dynamics impact synaptic degeneration in ALS

MJ Castellanos-Montiel, M Chaineau… - Frontiers in Cellular …, 2020 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that selectively affects
motor neurons (MNs) of the cortex, brainstem, and spinal cord. Several genes have been …

Modelling amyotrophic lateral sclerosis in rodents

TW Todd, L Petrucelli - Nature Reviews Neuroscience, 2022 - nature.com
The efficient study of human disease requires the proper tools, one of the most crucial of
which is an accurate animal model that faithfully recapitulates the human condition. The …

The actin cytoskeleton in SMA and ALS: how does it contribute to motoneuron degeneration?

N Hensel, P Claus - The Neuroscientist, 2018 - journals.sagepub.com
Amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA) are
neurodegenerative diseases with overlapping clinical phenotypes based on impaired …