Results 21 to 30 of about 160,996 (342)

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

Histological and functional benefit following transplantation of motor neuron progenitors to the injured rat spinal cord. [PDF]

PLoS ONE, 2010
Motor neuron loss is characteristic of cervical spinal cord injury (SCI) and contributes to functional deficit.In order to investigate the amenability of the injured adult spinal cord to motor neuron differentiation, we transplanted spinal cord injured ...
Sharyn L Rossi, Gabriel Nistor, Tanya Wyatt, Hong Zhen Yin, Aleksandra J Poole, John H Weiss, Matthew J Gardener, Sipke Dijkstra, David F Fischer, Hans S Keirstead   +9 more
doaj   +1 more source

Bisperoxovanadium promotes motor neuron survival and neuromuscular innervation in amyotrophic lateral sclerosis

Molecular Brain, 2021
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron (MN) disease, with no present cure. The progressive loss of MNs is the hallmark of ALS.
Junmei Wang, Lydia Tierney, Ranjeet Mann, Thomas Lonsway, Chandler L. Walker   +4 more
doaj   +1 more source

Stabilization of the survival motor neuron protein by ASK1 [PDF]

FEBS Letters, 2011
The survival motor neuron (SMN) is a spliceosomal snRNP-interacting protein that was initially identified as a defective molecule in spinal muscular atrophy (SMA). The disease severity of SMA is determined by SMN protein level. Here, we show that apoptosis signal-regulating kinase 1 (ASK1) stabilizes SMN protein by inhibiting SMN poly-ubiquitination ...
Jeong Eun Kwon, Eun‐Kyung Kim, Eui‐Ju Choi   +2 more
openalex   +4 more sources

Current evidence for treatment with nusinersen for spinal muscular atrophy : a systematic review [PDF]

, 2019
Recent discovery of nusinersen, an antisense oligonucleotide drug, has provided encouragement for improving treatment of spinal muscular atrophy. No therapeutic options currently exist for this autosomal recessive motor neuron disorder.
Meylemans, Antoon, De Bleecker, Jan
core   +1 more source

Association of the risk factor UNC13A with survival and upper motor neuron involvement in amyotrophic lateral sclerosis

Frontiers in Aging Neuroscience, 2023
BackgroundThe UNC13A gene is an established susceptibility locus for amyotrophic lateral sclerosis (ALS) and a determinant of shorter survival after disease onset, with up to 33.0 months difference in life expectancy for carriers of the rs12608932 risk ...
Arianna Manini, Arianna Manini, Valeria Casiraghi, Valeria Casiraghi, Alberto Brusati, Alberto Brusati, Alessio Maranzano, Alessio Maranzano, Francesco Gentile, Francesco Gentile, Eleonora Colombo, Ruggero Bonetti, Ruggero Bonetti, Silvia Peverelli, Sabrina Invernizzi, Davide Gentilini, Davide Gentilini, Stefano Messina, Federico Verde, Federico Verde, Barbara Poletti, Isabella Fogh, Claudia Morelli, Vincenzo Silani, Vincenzo Silani, Antonia Ratti, Antonia Ratti, Nicola Ticozzi, Nicola Ticozzi   +28 more
doaj   +1 more source

Survival Motor Neuron Protein in Motor Neurons Determines Synaptic Integrity in Spinal Muscular Atrophy [PDF]

Journal of Neuroscience, 2012
The inherited motor neuron disease spinal muscular atrophy (SMA) is caused by deficient expression of survival motor neuron (SMN) protein and results in severe muscle weakness. In SMA mice, synaptic dysfunction of both neuromuscular junctions (NMJs) and central sensorimotor synapses precedes motor neuron cell death.
Martinez, Tara L., Kong, Lingling, Wang, Xueyong, Osborne, Melissa A., Crowder, Melissa E., Van Meerbeke, James P., Yu, Xixi, Davis, Crystal, Wooley, Joe, Goldhamer, David J., Lutz, Cathleen M., Rich, Mark M., Sumner, Charlotte J.   +12 more
openaire   +3 more sources