- FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis
Department of Pathology and Cell Biology and Groupe de Recherche sur le Système Nerveux Central, University of Montreal, Montreal, Canada
PLoS Genet 7:e1002214. 2011
..Finally, overexpression of mutant SOD1 exacerbated the motor phenotype caused by overexpression of mutant FUS. Together our results indicate that TARDBP and FUS act in a pathogenic pathway that is independent of SOD1...
- Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo
Centre of Excellence in Neuromics, Department of Medicine, Universite de Montre al, Montreal, Quebec, Canada
Hum Mol Genet 19:671-83. 2010
- Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo
CRCHUM, Universite de Montreal, Montreal, QC, Canada
Neurobiol Dis 55:64-75. 2013
..Our results indicate that protein-folding homeostasis in the ER is an important target for therapeutic development in ALS and other TDP-43-related neurodegenerative diseases...
- Impaired proteasome function in sporadic amyotrophic lateral sclerosis
Department of Neurology Neurosurgery and Montreal Neurological Institute, McGill University, Montreal, Canada
Amyotroph Lateral Scler 13:367-71. 2012
..In conclusion, there are commonalities of findings in sporadic ALS patients and presymptomatic SOD1-G93A transgenic mice and these implicate inadequate proteasome function in the pathogenesis of both familial and sporadic ALS...
- Proteasomes remain intact, but show early focal alteration in their composition in a mouse model of amyotrophic lateral sclerosis
Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada
J Neurochem 105:2353-66. 2008
..Increase in detergent-insoluble, ubiquitinated proteins at P75 provided further evidence of stress on mechanisms of protein quality control in multiple cell types prior to significant motor neuron death...
- Proteasome activity or expression is not altered by activation of the heat shock transcription factor Hsf1 in cultured fibroblasts or myoblasts
David M Taylor
Montreal Neurological Institute, McGill University, Montreal, QC, Canada
Cell Stress Chaperones 10:230-41. 2005
..Thus, stress-induced or pharmacological activation of Hsf1 in mammalian cells would upregulate Hsps but not directly affect expression or activity of proteasomes...
- Als2 mRNA splicing variants detected in KO mice rescue severe motor dysfunction phenotype in Als2 knock-down zebrafish
Department of Medicine, Center for Excellence in Neuromics, CHUM Research Center, University of Montreal, Montreal, QC, Canada
Hum Mol Genet 17:2691-702. 2008
..Thus, we speculate that the newly identified Als2 mRNA species prevent the Als2 KO mice from developing severe neurodegenerative disease and might also regulate the severity of the motor neurons phenotype observed in ALS2 patients...
- Zebrafish models for the functional genomics of neurogenetic disorders
Department of Medicine, Universite de Montreal, Montreal, QC, Canada
Biochim Biophys Acta 1812:335-45. 2011
..This article is part of a Special Issue entitled Zebrafish Models of Neurological Diseases...
- Methylene blue protects against TDP-43 and FUS neuronal toxicity in C. elegans and D. rerio
Université de Montréal Hospital Research Centre, Montreal, Quebec, Canada
PLoS ONE 7:e42117. 2012
..Our results indicate that methylene blue can rescue toxic phenotypes associated with mutant TDP-43 and FUS including neuronal dysfunction and oxidative stress...
- Fishing for causes and cures of motor neuron disorders
Shunmoogum A Patten
Department of Neuroscience, FRQS Groupe de Recherche sur le Système Nerveux Central and CRCHUM, University of Montreal, Montreal, QC H3A 2B4, Canada
Dis Model Mech 7:799-809. 2014
..We present an overview of their potential usefulness in MND drug discovery, which is just beginning and holds much promise for future therapeutic development. ..
- TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
Center of Excellence in Neuromics, Centre Hospitalier de l Universite de Montreal, and Department of Medicine, University of Montreal, Montreal, Quebec H2L4MI, Canada
Nat Genet 40:572-4. 2008
..These findings further corroborate that TDP-43 is involved in ALS pathogenesis...
- In the swim of things: recent insights to neurogenetic disorders from zebrafish
Center for Excellence in Neuromics, Centre Hospitalier de l Université de Montréal CHUM Research Center and Department of Medicine, Universite de Montreal, Montreal, QC, Canada
Trends Genet 26:373-81. 2010
- Oxidized/misfolded superoxide dismutase-1: the cause of all amyotrophic lateral sclerosis?
Centre for Excellence in Neuromics, University of Montreal, the Centre Hospitalier de l Université de Montreal and Ste Justine Hospital, Montreal, Quebec, Canada
Ann Neurol 62:553-9. 2007
- Tryptophan 32 potentiates aggregation and cytotoxicity of a copper/zinc superoxide dismutase mutant associated with familial amyotrophic lateral sclerosis
David M Taylor
Department of Neurology and Neurosurgery, Montreal Neurological Institute and Sheldon Biotechnology Centre, McGill University, Montreal, Quebec H3A 2B4, Canada
J Biol Chem 282:16329-35. 2007
- Focal dysfunction of the proteasome: a pathogenic factor in a mouse model of amyotrophic lateral sclerosis
Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, 3801 University Street, Montreal, Quebec, Canada H3A 2B4
J Neurochem 89:1325-35. 2004
..It is concluded that impairment of the proteasome is an early event and contributes to ALS pathogenesis...