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Filters: Keyword is Amyotrophic Lateral Sclerosis and Author is J-P Julien [Clear All Filters]
Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1. Hum Mol Genet. 2013;22(19):3947-59.
Ablation of proliferating cells in the CNS exacerbates motor neuron disease caused by mutant superoxide dismutase. PLoS ONE. 2012;7(4):e34932.
Abnormal regenerative responses and impaired axonal outgrowth after nerve crush in TDP-43 transgenic mouse models of amyotrophic lateral sclerosis. J Neurosci. 2012;32(50):18186-95.
Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS. Proc Natl Acad Sci USA. 2012;109(14):5505-10.
Methylene blue administration fails to confer neuroprotection in two amyotrophic lateral sclerosis mouse models. Neuroscience. 2012;209:136-43.
ALS pathogenesis: recent insights from genetics and mouse models. Prog Neuropsychopharmacol Biol Psychiatry. 2011;35(2):363-9.
Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways. J Exp Med. 2011;208(12):2429-47.
Induction of protective immunity by vaccination with wild-type apo superoxide dismutase 1 in mutant SOD1 transgenic mice. J Neuropathol Exp Neurol. 2010;69(10):1044-56.
Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS. J Neurochem. 2010;113(5):1188-99.
Neuronal over-expression of chromogranin A accelerates disease onset in a mouse model of ALS. J Neurochem. 2010;115(5):1102-11.
Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. Nat Neurosci. 2010;13(11):1396-403.
Chromogranin B P413L variant as risk factor and modifier of disease onset for amyotrophic lateral sclerosis. Proc Natl Acad Sci USA. 2009;106(51):21777-82.
Ablation of proliferating microglia does not affect motor neuron degeneration in amyotrophic lateral sclerosis caused by mutant superoxide dismutase. J Neurosci. 2008;28(41):10234-44.
Als2 mRNA splicing variants detected in KO mice rescue severe motor dysfunction phenotype in Als2 knock-down zebrafish. Hum Mol Genet. 2008;17(17):2691-702.
ALS: astrocytes move in as deadly neighbors. Nat Neurosci. 2007;10(5):535-7.
Therapeutic effects of immunization with mutant superoxide dismutase in mice models of amyotrophic lateral sclerosis. Proc Natl Acad Sci USA. 2007;104(7):2495-500.
Therapeutic immunization with a glatiramer acetate derivative does not alter survival in G93A and G37R SOD1 mouse models of familial ALS. Neurobiol Dis. 2007;26(1):146-52.
Wild-type superoxide dismutase acquires binding and toxic properties of ALS-linked mutant forms through oxidation. J Neurochem. 2007;102(1):170-8.
Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat Neurosci. 2006;9(1):108-18.
Defective axonal transport of neurofilament proteins in neurons overexpressing peripherin. J Neurochem. 2006;98(3):926-38.
Transgenic mouse models of amyotrophic lateral sclerosis. Biochim Biophys Acta. 2006;1762(11-12):1013-24.