Results 21 to 30 of about 553,827 (305)
Onecut-dependent Nkx6.2 transcription factor expression is required for proper formation and activity of spinal locomotor circuits. [PDF]
, 2020 In the developing spinal cord, Onecut transcription factors control the diversification of motor neurons into distinct neuronal subsets by ensuring the maintenance of Isl1 expression during differentiation.
A Espana +36 more
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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 +3 more sources
Disease Models & Mechanisms, 2016 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
doaj +1 more source
Identification of a rhythmic firing pattern in the enteric nervous system that generates rhythmic electrical activity in smooth muscle [PDF]
, 2018 The enteric nervous system (ENS) contains millions of neurons essential for organization of motor behavior of the intestine. It is well established that the large intestine requires ENS activity to drive propulsive motor behaviors.
Brookes, Simon J +10 more
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Nature Communications, 2021 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
Brain Sciences, 2021 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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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 ...
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Nonsense mutations in alpha-II spectrin in three families with juvenile onset hereditary motor neuropathy [PDF]
, 2019 Distal hereditary motor neuropathies are a rare subgroup of inherited peripheral neuropathies hallmarked by a length-dependent axonal degeneration of lower motor neurons without significant involvement of sensory neurons.
Asselbergh, B +10 more
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Acta Neuropathologica Communications, 2020 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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Selective vulnerability of spinal and cortical motor neuron subpopulations in delta7 SMA mice. [PDF]
PLoS ONE, 2013 Loss of the survival motor neuron gene (SMN1) is responsible for spinal muscular atrophy (SMA), the most common inherited cause of infant mortality. Even though the SMA phenotype is traditionally considered as related to spinal motor neuron loss, it ...
Paolo d'Errico +9 more
doaj +1 more source