Publications
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, “Giant axonal neuropathy-associated gigaxonin mutations impair intermediate filament protein degradation.”, J Clin Invest, vol. 123, no. 5, pp. 1964-75, 2013.
, “Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1.”, Hum Mol Genet, vol. 22, no. 19, pp. 3947-59, 2013.
, “Neuroprotection through excitability and mTOR required in ALS motoneurons to delay disease and extend survival.”, Neuron, vol. 80, no. 1, pp. 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, pp. 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, pp. 5505-10, 2012.
, “Methylene blue administration fails to confer neuroprotection in two amyotrophic lateral sclerosis mouse models.”, Neuroscience, vol. 209, pp. 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, pp. 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, pp. 8501-8, 2012.
, “ALS pathogenesis: recent insights from genetics and mouse models.”, Prog Neuropsychopharmacol Biol Psychiatry, vol. 35, no. 2, pp. 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, pp. 2429-47, 2011.
, “Gigaxonin mutation analysis in patients with NIFID.”, Neurobiol Aging, vol. 32, no. 8, pp. 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, pp. 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, pp. 175-80, 2011.
, “Extracellular mutant SOD1 induces microglial-mediated motoneuron injury.”, Glia, vol. 58, no. 2, pp. 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, pp. 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, pp. 1188-99, 2010.
, “Neuronal over-expression of chromogranin A accelerates disease onset in a mouse model of ALS.”, J Neurochem, vol. 115, no. 5, pp. 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, pp. 2616-29, 2010.
, “Type III intermediate filament peripherin inhibits neuritogenesis in type II spiral ganglion neurons in vitro.”, Neurosci Lett, vol. 478, no. 2, pp. 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, pp. 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, pp. 245-50, 2010.
