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Impaired Synaptic Vesicle Release and Immaturity of Neuromuscular Junctions in Spinal Muscular Atrophy Mice
The motor neuron disease spinal muscular atrophy (SMA) causes profound muscle weakness that most often leads to early death. At autopsy, SMA is characterized by loss of motor neurons and muscleExpand
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Rescue of the spinal muscular atrophy phenotype in a mouse model by early postnatal delivery of SMN
Spinal muscular atrophy (SMA), the most common autosomal recessive neurodegenerative disease affecting children, results in impaired motor neuron function. Despite knowledge of the pathogenic role ofExpand
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In vivo visualization of pre- and postsynaptic changes during synapse elimination in reinnervated mouse muscle
  • M. Rich, J. Lichtman
  • Medicine, Biology
  • The Journal of neuroscience : the official…
  • 1 May 1989
Using a vital nerve terminal dye (4-Di-2-ASP) and fluorescently tagged alpha-bungarotoxin to stain postsynaptic acetylcholine (ACh) receptors, we viewed the same muscle fibers at multiple times inExpand
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Disruption of TrkB-Mediated Signaling Induces Disassembly of Postsynaptic Receptor Clusters at Neuromuscular Junctions
Neurotrophins and tyrosine receptor kinase (Trk) receptors are expressed in skeletal muscle, but it is unclear what functional role Trk-mediated signaling plays during postnatal life. Full-lengthExpand
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Early development of critical illness myopathy and neuropathy in patients with severe sepsis
Objectives: To characterize the prevalence, time of onset, and cause of neuromuscular dysfunction in patients with severe sepsis. Methods: We conducted a prospective cohort study in whichExpand
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Mice lacking the myotonic dystrophy protein kinase develop a late onset progressive myopathy
Myotonic dystrophy (DM) is an autosomal dominant disorder resulting from the expansion of a CTG repeat in the 3′ untranslated region of a putative protein kinase (DMPK). To elucidate the role of DMPKExpand
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Disruption of the endocytic protein HIP1 results in neurological deficits and decreased AMPA receptor trafficking
Huntingtin interacting protein 1 (HIP1) is a recently identified component of clathrin‐coated vesicles that plays a role in clathrin‐mediated endocytosis. To explore the normal function of HIP1 inExpand
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Temporal requirement for high SMN expression in SMA mice.
Spinal muscular atrophy (SMA) is caused by loss of the survival motor neuron 1 gene (SMN1) and retention of the SMN2 gene, resulting in reduced SMN. SMA mice can be rescued with high expression ofExpand
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Sensing and expressing homeostatic synaptic plasticity
Chronic changes in the level of neuronal activity (over a period of days) trigger compensatory changes in synaptic function that seem to contribute to the homeostatic restoration of neuronalExpand
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Direct muscle stimulation in acute quadriplegic myopathy.
We have previously found that muscle is electrically inexcitable in severe acute quadriplegic myopathy (AQM). In contrast, muscle retains normal electrical excitability in peripheral neuropathy. ToExpand
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