نتایج جستجو برای: neuron degeneration

تعداد نتایج: 122945  

Journal: :Neuron 2002
Bernadette H LaMonte Karen E Wallace Beth A Holloway Spencer S Shelly Jennifer Ascaño Mariko Tokito Thomas Van Winkle David S Howland Erika L.F Holzbaur

To test the hypothesis that inhibition of axonal transport is sufficient to cause motor neuron degeneration such as that observed in amyotrophic lateral sclerosis (ALS), we engineered a targeted disruption of the dynein-dynactin complex in postnatal motor neurons of transgenic mice. Dynamitin overexpression was found to disassemble dynactin, a required activator of cytoplasmic dynein, resulting...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2008
Koji Yamanaka Severine Boillee Elizabeth A Roberts Michael L Garcia Melissa McAlonis-Downes Oliver R Mikse Don W Cleveland Lawrence S B Goldstein

Dominant mutations in ubiquitously expressed superoxide dismutase (SOD1) cause familial ALS by provoking premature death of adult motor neurons. To test whether mutant damage to cell types beyond motor neurons is required for the onset of motor neuron disease, we generated chimeric mice in which all motor neurons and oligodendrocytes expressed mutant SOD1 at a level sufficient to cause fatal, e...

2013
Kensuke Ikenaka Kaori Kawai Masahisa Katsuno Zhe Huang Yue-Mei Jiang Yohei Iguchi Kyogo Kobayashi Tsubasa Kimata Masahiro Waza Fumiaki Tanaka Ikue Mori Gen Sobue

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of motor neurons. We previously showed that the expression of dynactin 1, an axon motor protein regulating retrograde transport, is markedly reduced in spinal motor neurons of sporadic ALS patients, although the mechanisms by which decreased dynactin 1 levels cause neurodegeneration ha...

2012

Clinical signs of upper motor neuron involvement are an essential observation to support the diagnosis of amyotrophic lateral sclerosis. However, clinical signs of upper motor neuron can be difficult to elicit in patients with motor neuron disease. One postulated reason for this problem is the presence of marked limb weakness and amyotrophy in motor neuron disease. This has been observed in pat...

Journal: :Frontiers in Physics 2021

The whole map of nematode connectomes provides important structural data for exploring the behavioral mechanism nematodes, but to further reveal functional importance and resilience pattern neurons, it is necessary effectively couple regulatory relationship between neurons their topology. Here, with a typical signal excitation function we propose model capture interacting because differential e...

Journal: :Neurology 2006
K A Josephs R C Petersen D S Knopman B F Boeve J L Whitwell J R Duffy J E Parisi D W Dickson

OBJECTIVE To examine the relationship between early clinical features, pathologies, and biochemistry of the frontotemporal lobar degenerations (FTLDs), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). METHODS The authors conducted pathologic reexamination with the most recent immunohistochemistry of all cases diagnosed with FTLD, PSP, and CBD between 1970 and 2004. T...

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