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
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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
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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
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Single copy/knock-in models of ALS SOD1 in C. elegans suggest loss and gain of function have different contributions to cholinergic and glutamatergic neurodegeneration [PDF]
, 2018 Mutations in Cu/Zn superoxide dismutase 1 (SOD1) lead to Amyotrophic Lateral Sclerosis (ALS), a neurodegenerative disease that disproportionately affects glutamatergic and cholinergic motor neurons.
Baskoylu, Saba N +10 more
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Reduced VGLUT2 expression increases motor neuron viability in Sod1G93A mice
Neurobiology of Disease, 2010 Glutamate-induced excitotoxicity has been suggested to influence pathogenesis in amyotrophic lateral sclerosis (ALS). Vesicular glutamate transporters (VGLUTs) are responsible for transport of glutamate into synaptic vesicles.
Hanna Wootz +4 more
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Military Medical Research, 2020 Background Motor neuron degeneration or loss in the spinal cord is the characteristic phenotype of motor neuron diseases or spinal cord injuries. Being proliferative and located near neurons, astrocytes are considered ideal cell sources for regenerating ...
An-Dong Zhao +4 more
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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
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Vascular regression precedes motor neuron loss in the FUS (1-359) ALS mouse model
Disease Models & Mechanisms, 2019 Amyotrophic lateral sclerosis (ALS) presents a poorly understood pathogenesis. Evidence from patients and mutant SOD1 mouse models suggests vascular damage may precede or aggravate motor dysfunction in ALS.
Martin Crivello +8 more
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Frontiers in Neuroscience, 2016 Motor neuron degeneration is the pathological hallmark of motor neuron diseases, a group of neurodegenerative disorders clinically manifested as muscle fasciculations and hyperreflexia, followed by paralysis, respiratory failure and death. Ample evidence
Rafael eLazo-Gómez, Ricardo eTapia
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TrkB signaling is required for postnatal survival of CNS neurons and protects hippocampal and motor neurons from axotomy-induced cell death [PDF]
, 1997 Newborn mice carrying targeted mutations in genes encoding neurotrophins or their signaling Trk receptors display severe neuronal deficits in the peripheral nervous system but not in the CNS. In this study, we show that trkB (¿/¿) mice have a significant
Alcántara Horrillo, Soledad +5 more
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