نتایج جستجو برای: motor neuron disorder
تعداد نتایج: 774054 فیلتر نتایج به سال:
Mutations in the RNA binding protein FUS cause amyotrophic lateral sclerosis (ALS), a fatal adult motor neuron disease. Decreased expression of SMN causes the fatal childhood motor neuron disorder spinal muscular atrophy (SMA). The SMN complex localizes in both the cytoplasm and nuclear Gems, and loss of Gems is a cellular hallmark of fibroblasts in patients with SMA. Here, we report that FUS a...
Frontotemporal dementia and motor neuron disease share clinical, genetic and pathological characteristics. Motor neuron disease develops in a proportion of patients with frontotemporal dementia, but the incidence, severity and functional significance of motor system dysfunction in patients with frontotemporal dementia has not been determined. Neurophysiological biomarkers have been developed to...
Amyotrophic lateral sclerosis (ALS) is recognized as a motor neuron disorder affecting the structure and function of the brain and neuromuscular system. Very little is known, however, that the nervous system degeneration is dependent on disease phenotypes of ALS. The purpose of this study was to determine the degree of brain white matter (WM) structure degeneration in four ALS patient groups ch...
background: children with attention deficit hyperactivity disorder/developmental coordination disorder (adhd/dcd) suffer from problems associated with gross and fine motor skills. there is no effective pharmacological therapy for such patients. we aimed to assess the impact of methylphenidate (mph) on motor performance of children with adhd/dcd. methods: in this double-blind placebo-controlled,...
ALS is a fatal paralytic disorder characterized by a progressive loss of spinal cord motor neurons. Herein, we show that NADPH oxidase, the main reactive oxygen species-producing enzyme during inflammation, is activated in spinal cords of ALS patients and in spinal cords in a genetic animal model of this disease. We demonstrate that inactivation of NADPH oxidase in ALS mice delays neurodegenera...
Received 24 December 2010 Revised 24 January 2011 Accepted 25 January 2011 Early online 27 January 2011 Print 31 January 2011 Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) is a relentlessly progressive neurological disorder causing peripheral muscular weakness and resultant respiratory failure. In this article, we report a case of ALS with chronic obstructive pul...
The topographic assembly of neural circuits is dependent upon the generation of specific neuronal subtypes, each subtype displaying unique properties that direct the formation of selective connections with appropriate target cells. Studies of motor neuron development in the spinal cord have begun to elucidate the molecular mechanisms involved in controlling motor projections. In this review, we...
Amyotrophic lateral sclerosis (ALS) is a fatal and rapidly progressing motor neuron disease. Astrocytic factors are known to contribute to motor neuron degeneration and death in ALS. However, the role of astrocyte in promoting motor neuron protein aggregation, a disease hallmark of ALS, remains largely unclear. Here, using culture models of human motor neurons and primary astrocytes of differen...
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