Results 21 to 30 of about 390,159 (364)
Cell Reports, 2016 In spinal muscular atrophy, a neurodegenerative disease caused by ubiquitous deficiency in the survival motor neuron (SMN) protein, sensory-motor synaptic dysfunction and increased excitability precede motor neuron (MN) loss.
Christian M. Simon +5 more
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Frontiers in Molecular Neuroscience, 2021 The past decade has seen a rapid acceleration in the discovery of new genetic causes of ALS, with more than 20 putative ALS-causing genes now cited. These genes encode proteins that cover a diverse range of molecular functions, including free radical ...
Flora Cheng +28 more
doaj +1 more source
Electrophysiological studies in healthy subjects involving caffeine [PDF]
, 2010 Copyright ©2012 IOS Press All rights reserved.We review the electrophysiological studies concerning the effects of caffeine on muscle, lower and upper motor neuron excitability and cognition.
Carvalho, Mamede +2 more
core +1 more source
BMJ, 2011 Diane Sackett experienced the first symptoms of motor neurone disease in 2004, and died in September 2009. Diane’s husband, Brian, was her main carer.
Mohamed Sakel, Brian Sackett
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Postgraduate Medical Journal, 2002 Abstract Motor neurone disease (MND), or amyotrophic lateral sclerosis (ALS), is a neurodegenerative disorder of unknown aetiology. Progressive motor weakness and bulbar dysfunction lead to premature death, usually from respiratory failure. Confirming the diagnosis may initially be difficult until the full clinical features are manifest.
openaire +4 more sources
Current evidence for treatment with nusinersen for spinal muscular atrophy : a systematic review [PDF]
, 2019 Recent discovery of nusinersen, an antisense oligonucleotide drug, has provided encouragement for improving treatment of spinal muscular atrophy. No therapeutic options currently exist for this autosomal recessive motor neuron disorder.
Meylemans, Antoon, De Bleecker, Jan
core +1 more source
FUS-SMN Protein Interactions Link the Motor Neuron Diseases ALS and SMA [PDF]
, 2012 SummaryMutations 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).
Battle +30 more
core +2 more sources
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
Motor Neurons that Multitask [PDF]
Neuron, 2012 Animals use a form of sensory feedback termed proprioception to monitor their body position and modify the motor programs that control movement. In this issue of Neuron, Wen et al. (2012) provide evidence that a subset of motor neurons function as proprioceptors in C.
openaire +3 more sources
cDNA that encodes active agrin [PDF]
, 1992 Agrin is thought to mediate the motor neuron-induced aggregation of AChRs and AChE on the surface of muscle fibers at neuromuscular junctions. We have isolated a cDNA from a chick brain library that, based on sequence homology and expression experiments,
Bolander +42 more
core +2 more sources