Results 31 to 40 of about 2,514,411 (397)
HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients
Nature Communications, 2017 Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder due to selective loss of motor neurons (MNs). Mutations in the fused in sarcoma (FUS) gene can cause both juvenile and late onset ALS.
Wenting Guo +25 more
semanticscholar +1 more source
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
doaj +1 more source
Effects of lipopolysaccharide-induced inflammation on expression of growth-associated genes by corticospinal neurons [PDF]
, 2006 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
core +2 more sources
Nature Communications, 2018 Mutations in C9ORF72 are the most common cause of familial amyotrophic lateral sclerosis (ALS). Here, through a combination of RNA-Seq and electrophysiological studies on induced pluripotent stem cell (iPSC)-derived motor neurons (MNs), we show that ...
B. Selvaraj +33 more
semanticscholar +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 +2 more sources
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
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
doaj +1 more source
Prepontine non-giant neurons drive flexible escape behavior in zebrafish [PDF]
, 2019 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
core +2 more sources
Comparing Offline Decoding Performance in Physiologically Defined Neuronal Classes [PDF]
, 2016 Objective: Recently, several studies have documented the presence of a bimodal distribution of spike waveform widths in primary motor cortex. Although narrow and wide spiking neurons, corresponding to the two modes of the distribution, exhibit different ...
Best, Matthew D. +5 more
core +2 more sources
Cell cycle inhibitors protect motor neurons in an organoid model of Spinal Muscular Atrophy
Cell Death and Disease, 2018 Spinal Muscular Atrophy (SMA) is caused by genetic mutations in the SMN1 gene, resulting in drastically reduced levels of Survival of Motor Neuron (SMN) protein.
Jin-Hui Hor +10 more
semanticscholar +1 more source