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 …

Progesterone neuroprotection in traumatic CNS injury and motoneuron degeneration

AF De Nicola, F Labombarda, MCG Deniselle… - Frontiers in …, 2009 - Elsevier
Studies on the neuroprotective and promyelinating effects of progesterone in the nervous
system are of great interest due to their potential clinical connotations. In peripheral …

Neuroprotective effects of the Sigma-1 receptor (S1R) agonist PRE-084, in a mouse model of motor neuron disease not linked to SOD1 mutation

M Peviani, E Salvaneschi, L Bontempi, A Petese… - Neurobiology of …, 2014 - Elsevier
The identification of novel molecular targets crucially involved in motor neuron
degeneration/survival is a necessary step for the development of hopefully more effective …

Requirement of the human GARP complex for mannose 6-phosphate-receptor-dependent sorting of cathepsin D to lysosomes

FJ Pérez-Victoria, GA Mardones… - Molecular biology of the …, 2008 - Am Soc Cell Biol
The biosynthetic sorting of acid hydrolases to lysosomes relies on transmembrane,
mannose 6-phosphate receptors (MPRs) that cycle between the TGN and endosomes …

The wobbler mouse, an ALS animal model

JM Moser, P Bigini, T Schmitt-John - Molecular Genetics and Genomics, 2013 - Springer
This review article is focused on the research progress made utilizing the wobbler mouse as
animal model for human motor neuron diseases, especially the amyotrophic lateral sclerosis …

Toll-like receptors in central nervous system injury and disease: a focus on the spinal cord

A Heiman, A Pallottie, RF Heary, S Elkabes - Brain, behavior, and immunity, 2014 - Elsevier
Toll-like receptors (TLRs) are best known for recognizing pathogens and initiating an innate
immune response to protect the host. However, they also detect tissue damage and induce …

Mouse behavioural analysis in systems biology

P Van Meer, J Raber - Biochemical Journal, 2005 - portlandpress.com
Molecular techniques allowing in vivo modulation of gene expression have provided unique
opportunities and challenges for behavioural studies aimed at understanding the function of …

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
Numerous mutations that impair retrograde membrane trafficking between endosomes and
the Golgi apparatus lead to neurodegenerative diseases. For example, mutations in the …

Reduced GABAergic inhibition explains cortical hyperexcitability in the wobbler mouse model of ALS

JL Nieto-Gonzalez, J Moser, M Lauritzen… - Cerebral …, 2011 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of the central
nervous system. Symptomatic and presymptomatic ALS patients demonstrate cortical …

Role of GARP vesicle tethering complex in Golgi physiology

A Khakurel, VV Lupashin - International journal of molecular sciences, 2023 - mdpi.com
The Golgi associated retrograde protein complex (GARP) is an evolutionarily conserved
component of Golgi membrane trafficking machinery that belongs to the Complexes …