Results 11 to 20 of about 159,037 (298)

Overexpression of survival motor neuron improves neuromuscular function and motor neuron survival in mutant SOD1 mice

Neurobiology of Aging, 2014
Spinal muscular atrophy results from diminished levels of survival motor neuron (SMN) protein in spinal motor neurons. Low levels of SMN also occur in models of amyotrophic lateral sclerosis (ALS) caused by mutant superoxide dismutase 1 (SOD1) and genetic reduction of SMN levels exacerbates the phenotype of transgenic SOD1(G93A) mice.
Turner, B, Turner, B, Alfazema, N, Sheean, R, Sheean, R, Sleigh, J, Sleigh, J, Davies, K, Horne, M, Horne, M, Talbot, K, Talbot, K   +11 more
openaire   +8 more sources

A high-throughput genome-wide RNAi screen identifies modifiers of survival motor neuron protein. [PDF]

Cell Rep, 2021
McCormack NM, Abera MB, Arnold ES, Gibbs RM, Martin SE, Buehler E, Chen YC, Chen L, Fischbeck KH, Burnett BG.   +9 more
europepmc   +2 more sources

Gamma motor neurons survive and exacerbate alpha motor neuron degeneration in ALS [PDF]

Proceedings of the National Academy of Sciences, 2016
Significance Clinical and pathological hallmarks shared by various familial and sporadic forms of amyotrophic lateral sclerosis (ALS) suggest common underlying mechanisms of disease. Using a series of ALS mouse models, we demonstrate that one shared feature of ALS is the selective sparing of gamma motor neurons (γ-MNs), which ...
Melanie, Lalancette-Hebert, Aarti, Sharma, Alexander K, Lyashchenko, Neil A, Shneider   +3 more
openaire   +2 more sources

Sensory neurons do not induce motor neuron loss in a human stem cell model of spinal muscular atrophy. [PDF]

PLoS ONE, 2014
Spinal muscular atrophy (SMA) is an autosomal recessive disorder leading to paralysis and early death due to reduced SMN protein. It is unclear why there is such a profound motor neuron loss, but recent evidence from fly and mouse studies indicate that ...
Andrew J Schwab, Allison D Ebert
doaj   +1 more source

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, A Espana, A Gow, A Harris, A Roy, A Wilzen, AE Talpalar, AF Ribeiro Jr., C Francius, C Landry, DC Lu, E Audouard, E Audouard, E Palmesino, F Clotman, F Clotman, FJ Stam, FP Lemaigre, HS Rhee, I Laudadio, J Cai, KU Kabayiza, L Landmesser, L Landmesser, M Sander, M Toch, M Zuchner, NR Lorente De, O Kjaerulff, P Jacquemin, P Jacquemin, P Whelan, S Velasco, TM Jessell, V Vanhorenbeeck, VJ Lannoy, W Wang   +36 more
core   +1 more source

Dysregulation of Rac or Rho elicits death of motor neurons and activation of these GTPases is altered in the G93A mutant hSOD1 mouse model of amyotrophic lateral sclerosis

Neurobiology of Disease, 2020
Rho GTPases play a central role in neuronal survival; however, the antagonistic relationship between Rac and Rho in the regulation of motor neuron survival remains poorly defined.
Trisha R. Stankiewicz, Claudia Pena, Ron J. Bouchard, Daniel A. Linseman   +3 more
doaj   +1 more source

Motor defects in a Drosophila model for spinal muscular atrophy result from SMN depletion during early neurogenesis.

PLoS Genetics, 2022
Spinal muscular atrophy (SMA) is the most common autosomal recessive neurodegenerative disease, and is characterised by spinal motor neuron loss, impaired motor function and, often, premature death.
Stuart J Grice, Ji-Long Liu
doaj   +1 more source

Bcl-xL Promotes the Survival of Motor Neurons Derived from Neural Stem Cells

Biology, 2023
Neural stem cell (NSC) transplantation creates new hope for the treatment of neurodegenerative disorders by direct differentiation into neurons. However, this technique is limited by poor survival and functional neuron deficiency. In this research study,
Yunqin Wu, Xiaohua Peng, Song Ang, Yue Gao, Yue Chi, Jinling Wang, Chengcheng Tang, Xiaoqing Zhou, Yanxian Feng, Kun Zhang, Qingjian Zou, Min Chen   +11 more
doaj   +1 more source

Genetic deficiency of GABA differentially regulates respiratory and non-respiratory motor neuron development. [PDF]

PLoS ONE, 2013
Central nervous system GABAergic and glycinergic synaptic activity switches from postsynaptic excitation to inhibition during the stage when motor neuron numbers are being reduced, and when synaptic connections are being established onto and by motor ...
Matthew J Fogarty, Karen L Smallcombe, Yuchio Yanagawa, Kunihiko Obata, Mark C Bellingham, Peter G Noakes   +5 more
doaj   +1 more source

Calcineurin inhibition enhances motor neuron survival following injury [PDF]

Journal of Cellular and Molecular Medicine, 2010
AbstractThe immunosuppressive agents cyclosporin A (CsA) and FK‐506 have previously been shown to exhibit neurotrophic and neuroprotective propertiesin vivo. Given that significant clinical expertise exists for both drugs, they represent an attractive starting point for treatment of acute neural injuries.
Hui, Kelvin KW, Liadis, Nicole, Robertson, Jennifer, Kanungo, Anish, Henderson, Jeffrey T   +4 more
openaire   +3 more sources