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Filtres: Author is J-P Julien [Enlever les filtres]
Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells. Mol Brain. 2015;8(1):71.
Adeno-associated virus-mediated delivery of a recombinant single-chain antibody against misfolded superoxide dismutase for treatment of amyotrophic lateral sclerosis. Mol Ther. 2014;22(3):498-510.
Interaction of transactive response DNA binding protein 43 with nuclear factor κB in mild cognitive impairment with episodic memory deficits. Acta Neuropathol Commun. 2014;2:37.
Loss of glial neurofascin155 delays developmental synapse elimination at the neuromuscular junction. J Neurosci. 2014;34(38):12904-18.
Axonal transport deficits and neurodegenerative diseases. Nat Rev Neurosci. 2013;14(3):161-76.
Giant axonal neuropathy-associated gigaxonin mutations impair intermediate filament protein degradation. J Clin Invest. 2013;123(5):1964-75.
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.
Local electronic structure and Fano interference in tunneling into a Kondo hole system. Phys Rev Lett. 2012;108(18):186401.
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.
Normal role of the low-molecular-weight neurofilament protein in mitochondrial dynamics and disruption in Charcot-Marie-Tooth disease. FASEB J. 2012;26(3):1194-203.
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.
Gigaxonin mutation analysis in patients with NIFID. Neurobiol Aging. 2011;32(8):1528-9.
Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset. PLoS ONE. 2011;6(7):e22031.
The myosin Va head domain binds to the neurofilament-L rod and modulates endoplasmic reticulum (ER) content and distribution within axons. PLoS ONE. 2011;6(2):e17087.
Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments. Brain. 2011;134(Pt 9):2610-26.
Sensorimotor and cognitive function of a NEFL(P22S) mutant model of Charcot-Marie-Tooth disease type 2E. Behav Brain Res. 2011;219(2):175-80.
Extracellular mutant SOD1 induces microglial-mediated motoneuron injury. Glia. 2010;58(2):231-43.
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.