Results 31 to 40 of about 390,159 (364)
Cell Reports, 2013 Spinal muscular atrophy (SMA) is a neurodegenerative disease caused by a deficiency in the survival motor neuron (SMN) protein. SMN mediates the assembly of spliceosomal small nuclear ribonucleoproteins (snRNPs) and possibly other RNPs.
Sarah Tisdale +7 more
doaj +1 more source
Heavy metals in locus ceruleus and motor neurons in motor neuron disease [PDF]
Acta Neuropathologica Communications, 2013 The causes of sporadic amyotrophic lateral sclerosis (SALS) and other types of motor neuron disease (MND) remain largely unknown. Heavy metals have long been implicated in MND, and it has recently been shown that inorganic mercury selectively enters human locus ceruleus (LC) and motor neurons. We therefore used silver nitrate autometallography (AMG) to
Roger Pamphlett, Stephen Kum Jew
openaire +3 more sources
Notch Signaling Regulates Motor Neuron Differentiation of Human Embryonic Stem Cells [PDF]
, 2015 In the pMN domain of the spinal cord, Notch signaling regulates the balance between motor neuron differentiation and maintenance of the progenitor state for later oligodendrocyte differentiation.
Ben‐shushan, Etti +2 more
core +1 more source
Postgraduate Medical Journal, 1962 THE story of motor neurone disease goes back more than a hundred years, to the latter half of the igth century, and the days of the great clinical neurologists of France. Of the many famous names linked to this story three are pre-eminent: Charcot-physician and neuropathologist, and great teacher, who became even more renowned for his studies of ...
openaire +3 more sources
ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
Nature Communications, 2016 The mechanism by which FUS mutations cause familial ALS remains unclear. Here, the authors use mouse transgenic models to show that a toxic gain-of-function underlies motor neuron degeneration, and that the toxicity of mutant FUS does not depend on a ...
Aarti Sharma +9 more
doaj +1 more source
Neuronal function of Tbx20 conserved from nematodes to vertebrates [PDF]
, 2008 The Tbx20 orthologue, mab-9, is required for development of the Caenorhabditis elegans hindgut, whereas several vertebrate Tbx20 genes promote heart development.
Aslam, Sobia +8 more
core +1 more source
Modulation of cortical motor outputs by the symbolic meaning of visual stimuli. [PDF]
, 2010 The observation of an action modulates motor cortical outputs in specific ways, in part through mediation of the mirror neuron system. Sometimes we infer a meaning to an observed action based on integration of the actual percept with memories.
Aziz-Zadeh +28 more
core +1 more source
Biology Open, 2013 Summary Amyotrophic Lateral Sclerosis (ALS) is a motor neuron degenerative disease characterized by a progressive, and ultimately fatal, muscle paralysis. The human VAMP-Associated Protein B (hVAPB) is the causative gene of ALS type 8.
Mario Sanhueza +3 more
doaj +1 more source
Tracheostomy in motor neurone disease [PDF]
Practical Neurology, 2019 Tracheostomy-associated ventilation for the respiratory insufficiency caused by amyotrophic lateral sclerosis (motor neurone disease (MND)) is a complex issue with practical, ethical and economic dimensions. This article considers the current prevalence of tracheostomy in MND, the evidence for its benefit both for survival and quality of life, and the ...
Martin R Turner +5 more
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Making tau amyloid models in vitro: a crucial and underestimated challenge
FEBS Letters, EarlyView.This review highlights the challenges of producing in vitro amyloid assemblies of the tau protein. We review how accurately the existing protocols mimic tau deposits found in the brain of patients affected with tauopathies. We discuss the important properties that should be considered when forming amyloids and the benchmarks that should be used to ...
Julien Broc, Clara Piersson, Yann Fichou
wiley +1 more source