Inhibition of sphingolipid synthesis improves outcomes and survival in GARP mutant wobbler mice, a model of motor neuron degeneration

CS Petit, JJ Lee, S Boland, S Swarup… - Proceedings of the …, 2020 - National Acad Sciences
… -treated wobbler mice. Astrocytes have been implicated as key cell types in modulating
neurodegeneration … To assess the role of astrocytes in neurodegeneration of wobbler mice, we …

Early Signs of Neuroinflammation in the postnatal wobbler mouse model of amyotrophic lateral sclerosis

M Meyer, A Lima, MCG Deniselle… - Cellular and Molecular …, 2023 - Springer
… a reactive response to neurodegeneration, although other … Wobbler mouse model of sALS
provides important information regarding the role of different cells in this disease. Wobblers

Neuroprotective effects of testosterone in male wobbler mouse, a model of amyotrophic lateral sclerosis

A Lara, I Esperante, M Meyer, P Liere… - Molecular …, 2021 - Springer
… role of testosterone in neurodegeneration of the Wobbler mouse. Wobblers bear a spontaneous
Wobbler mice show a spontaneous mutation of the gene that encodes for the Vps 54 …

Little helpers or mean rogue—role of microglia in animal models of amyotrophic lateral sclerosis

H Cihankaya, C Theiss, V Matschke - International journal of molecular …, 2021 - mdpi.com
… M2-type cytokines in wobbler mice. However, the histological and neurological outcomes
of wobbler mice bring to mind the fact that neurodegenerative cytokines released by microglia …

Steroid profiling in male Wobbler mouse, a model of amyotrophic lateral sclerosis

MC Gonzalez Deniselle, P Liere, A Pianos… - …, 2016 - academic.oup.com
… during neurodegenerative diseases (4, 19). Thus, the neuroprotective effects of PROG have
been shown in models of injured nervous system and in some neurodegenerative diseases (…

Excitatory-inhibitory imbalance in the brain of the wobbler mouse model of amyotrophic lateral sclerosis substantiated by riluzole and diazepam

SR Andreasen, CJ Lundbye, TB Christensen… - Neuroscience …, 2017 - Elsevier
… ) is a devastating neurodegenerative disease affecting upper … that it may contribute to the
neurodegeneration in familial ALS [39… The wobbler mouse model of ALS displays several of the …

[HTML][HTML] Dysregulated expression and distribution of Kif5α in neurites of wobbler motor neurons

K Kürten, AC Gude, ASC Epplen, J Stein… - Neural regeneration …, 2023 - journals.lww.com
… mice and ALS patients, leading to neurodegeneration. A recent study on a Charcot Marie
Tooth model revealed that compensation for the loss of Kif5α dependent cargo inhibited axonal …

ROS scavengers decrease γH2ax spots in motor neuronal nuclei of ALS model mice in vitro

M Junghans, F John, H Cihankaya… - Frontiers in Cellular …, 2022 - frontiersin.org
… ) is an incurable neurodegenerative disease characterized by the … breaks (DSBs) in the ALS
model of the Wobbler mouse. … phenotype of the homozygous Wobbler mouse shows a head …

MicroRNA expression in animal models of amyotrophic lateral sclerosis and potential therapeutic approaches

B Martinez, PV Peplow - Neural regeneration research, 2022 - journals.lww.com
… The wobbler mouse model The wobbler mouse was the first proposed mouse model of
ALS … gray matter of the ventral horn, the site of neurodegeneration. The number of miR-9-positive …

Elevated NLRP3 Inflammasome Activation Is Associated with Motor Neuron Degeneration in ALS

H Cihankaya, V Bader, KF Winklhofer, M Vorgerd… - Cells, 2024 - mdpi.com
… ) is a fatal neurodegenerative disease characterized by motor … interplay of inflammation and
neurodegeneration in ALS. This … Since the wobbler mouse shows motor neuron degeneration…