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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.
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Large-scale Spatiotemporal Spike Patterning Consistent with Wave Propagation in Motor Cortex [PDF]
Aggregate signals in cortex are known to be spatiotemporally organized as propagating waves across the cortical surface, but it remains unclear whether the same is true for spiking activity in individual neurons.
Best, Matthew D. +6 more
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Effects of lipopolysaccharide-induced inflammation on expression of growth-associated genes by corticospinal neurons [PDF]
Background: Inflammation around cell bodies of primary sensory neurons and retinal ganglion cells enhances expression of neuronal growth-associated genes and stimulates axonal regeneration.
Anderson, PN +5 more
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Spinal muscular atrophy (SMA), characterized by specific degeneration of spinal motor neurons, is caused by mutations in the survival of motor neuron 1, telomeric (SMN1) gene and subsequent decreased levels of functional SMN. How the deficiency of SMN, a
Chong-Chong Xu +4 more
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Here the authors show in a model of ALS that motor neurons receive stronger inhibitory synaptic inputs than slow motor neurons, and disease progression is associated with specific loss of inhibitory synapses onto fast motor neurons.
Ilary Allodi +4 more
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Differential NPY-Y1 Receptor Density in the Motor Cortex of ALS Patients and Familial Model of ALS
Destabilization of faciliatory and inhibitory circuits is an important feature of corticomotor pathology in amyotrophic lateral sclerosis (ALS). While GABAergic inputs to upper motor neurons are reduced in models of the disease, less understood is the ...
Courtney M. Clark +5 more
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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 ...
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Prepontine non-giant neurons drive flexible escape behavior in zebrafish [PDF]
Many species execute ballistic escape reactions to avoid imminent danger. Despite fast reaction times, responses are often highly regulated, reflecting a trade-off between costly motor actions and perceived threat level.
Bergeron, S. +4 more
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DNA damage is implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS). However, relationships between DNA damage accumulation, DNA damage response (DDR), and upper and lower motor neuron vulnerability in human ALS are unclear; furthermore ...
Byung Woo Kim +3 more
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Aberrant axon initial segment plasticity and intrinsic excitability of ALS hiPSC motor neurons
Summary: Dysregulated neuronal excitability is a hallmark of amyotrophic lateral sclerosis (ALS). We sought to investigate how functional changes to the axon initial segment (AIS), the site of action potential generation, could impact neuronal ...
Peter Harley +9 more
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