Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis. [PDF]
, 2013 Oligodendrocytes associate with axons to establish myelin and provide metabolic support to neurons. In the spinal cord of amyotrophic lateral sclerosis (ALS) mice, oligodendrocytes downregulate transporters that transfer glycolytic substrates to neurons ...
Bergles, Dwight E +7 more
core +2 more sources
Structural Properties of the Caenorhabditis elegans Neuronal Network [PDF]
, 2010 Despite recent interest in reconstructing neuronal networks, complete wiring diagrams on the level of individual synapses remain scarce and the insights into function they can provide remain unclear.
Chen, Beth L. +4 more
core +7 more sources
Survival motor neuron protein facilitates assembly of stress granules [PDF]
FEBS Letters, 2004 The survival motor neuron (SMN) protein forms cytoplasmic granules when overexpressed. We report here that SMN co‐localizes with TIA‐1/R and G3BP, protein assemblers of stress granules (SGs), and that SMN is co‐immunoprecipitated with TIA‐1/R, suggesting that SMN granules are SGs.
Hua, Yimin, Zhou, Jianhua
openaire +2 more sources
Sephin1 reduces TDP-43 cytoplasmic mislocalization and improves motor neuron survival in ALS models
Life Science AllianceSephin1, an UPR pathway modulator, mitigates TDP-43 cytoplasmic localization and toxicity in ALS motor neurons, improving their survival and showing potential as a therapeutic for ALS.
Emmanuelle Abgueguen +16 more
doaj +1 more source
Altered Metabolic Profiles Associate with Toxicity in SOD1G93A Astrocyte-Neuron Co-Cultures
Scientific Reports, 2017 Non-cell autonomous processes involving astrocytes have been shown to contribute to motor neuron degeneration in amyotrophic lateral sclerosis. Mutant superoxide dismutase 1 (SOD1G93A) expression in astrocytes is selectively toxic to motor neurons in co ...
Gabriel N. Valbuena +4 more
doaj +1 more source
Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons. [PDF]
PLoS ONE, 2013 In the inherited childhood neuromuscular disease spinal muscular atrophy (SMA), lower motor neuron death and severe muscle weakness result from the reduction of the ubiquitously expressed protein survival of motor neuron (SMN).
Ximena Paez-Colasante +5 more
doaj +1 more source
Viral delivery of antioxidant genes as a therapeutic strategy in experimental models of amyotrophic lateral sclerosis. [PDF]
, 2013 Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with no effective treatment to date. Despite its multi-factorial aetiology, oxidative stress is hypothesized to be one of the key pathogenic mechanisms.
Azzouz, M +6 more
core +1 more source
Neurobiology of Disease, 2007 Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the selective loss of motor neurons. Stress activated protein kinases (SAPK) have been suggested to play a role in the pathogenesis of ALS.
Maarten Dewil +3 more
doaj +1 more source
Ca2+-activated K+ channels modulate microglia affecting motor neuron survivalin hSOD1G93A mice [PDF]
, 2018 Recent studies described a critical role for microglia in amyotrophic lateral sclerosis (ALS), where these CNS-resident immune cells participate in the establishment of an inflammatory microenvironment that contributes to motor neuron degeneration ...
Antonangeli, Fabrizio +13 more
core +2 more sources
Predicting survival in frontotemporal dementia with motor neuron disease [PDF]
Neurology, 2011 To determine whether clinical and demographic features are associated with prognosis in patients with frontotemporal dementia and motor neuron disease (FTD-MND).This was a case series of FTD-MND categorized according to behavioral- or language-dominant symptoms at presentation and throughout the disease course.
E A, Coon +4 more
openaire +2 more sources