Publications
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Filtres: Author is J-P Julien [Enlever les filtres]
, « Giant axonal neuropathy-associated gigaxonin mutations impair intermediate filament protein degradation. », J Clin Invest, vol. 123, nᵒ 5, p. 1964-75, 2013.
, « Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1. », Hum Mol Genet, vol. 22, nᵒ 19, p. 3947-59, 2013.
, « Neuroprotection through excitability and mTOR required in ALS motoneurons to delay disease and extend survival. », Neuron, vol. 80, nᵒ 1, p. 80-96, 2013.
, « Ablation of proliferating cells in the CNS exacerbates motor neuron disease caused by mutant superoxide dismutase. », PLoS One, vol. 7, nᵒ 4, p. e34932, 2012.
, « Abnormal regenerative responses and impaired axonal outgrowth after nerve crush in TDP-43 transgenic mouse models of amyotrophic lateral sclerosis. », J Neurosci, vol. 32, nᵒ 50, p. 18186-95, 2012.
, « Local electronic structure and Fano interference in tunneling into a Kondo hole system. », Phys Rev Lett, vol. 108, nᵒ 18, p. 186401, 2012.
, « Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS. », Proc Natl Acad Sci U S A, vol. 109, nᵒ 14, p. 5505-10, 2012.
, « Methylene blue administration fails to confer neuroprotection in two amyotrophic lateral sclerosis mouse models. », Neuroscience, vol. 209, p. 136-43, 2012.
, « Normal role of the low-molecular-weight neurofilament protein in mitochondrial dynamics and disruption in Charcot-Marie-Tooth disease. », FASEB J, vol. 26, nᵒ 3, p. 1194-203, 2012.
, « Peripherin is a subunit of peripheral nerve neurofilaments: implications for differential vulnerability of CNS and peripheral nervous system axons. », J Neurosci, vol. 32, nᵒ 25, p. 8501-8, 2012.
, « ALS pathogenesis: recent insights from genetics and mouse models. », Prog Neuropsychopharmacol Biol Psychiatry, vol. 35, nᵒ 2, p. 363-9, 2011.
, « Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways. », J Exp Med, vol. 208, nᵒ 12, p. 2429-47, 2011.
, « Gigaxonin mutation analysis in patients with NIFID. », Neurobiol Aging, vol. 32, nᵒ 8, p. 1528-9, 2011.
, « Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset. », PLoS One, vol. 6, nᵒ 7, p. e22031, 2011.
, « The myosin Va head domain binds to the neurofilament-L rod and modulates endoplasmic reticulum (ER) content and distribution within axons. », PLoS One, vol. 6, nᵒ 2, p. e17087, 2011.
, « Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments. », Brain, vol. 134, nᵒ Pt 9, p. 2610-26, 2011.
, « Sensorimotor and cognitive function of a NEFL(P22S) mutant model of Charcot-Marie-Tooth disease type 2E. », Behav Brain Res, vol. 219, nᵒ 2, p. 175-80, 2011.
, « Extracellular mutant SOD1 induces microglial-mediated motoneuron injury. », Glia, vol. 58, nᵒ 2, p. 231-43, 2010.
, « Induction of protective immunity by vaccination with wild-type apo superoxide dismutase 1 in mutant SOD1 transgenic mice. », J Neuropathol Exp Neurol, vol. 69, nᵒ 10, p. 1044-56, 2010.
, « 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, vol. 113, nᵒ 5, p. 1188-99, 2010.
, « Neuronal over-expression of chromogranin A accelerates disease onset in a mouse model of ALS. », J Neurochem, vol. 115, nᵒ 5, p. 1102-11, 2010.
, « Reversal of neuropathy phenotypes in conditional mouse model of Charcot-Marie-Tooth disease type 2E. », Hum Mol Genet, vol. 19, nᵒ 13, p. 2616-29, 2010.
, « Type III intermediate filament peripherin inhibits neuritogenesis in type II spiral ganglion neurons in vitro. », Neurosci Lett, vol. 478, nᵒ 2, p. 51-5, 2010.
, « Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. », Nat Neurosci, vol. 13, nᵒ 11, p. 1396-403, 2010.
, « Wild-type human SOD1 overexpression does not accelerate motor neuron disease in mice expressing murine Sod1 G86R. », Neurobiol Dis, vol. 40, nᵒ 1, p. 245-50, 2010.
