Results 21 to 30 of about 159,037 (298)

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

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

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

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

Neuronal-Specific Roles of the Survival Motor Neuron Protein [PDF]

Journal of Neuropathology and Experimental Neurology, 2006
Despite recent data on the cellular function of the survival motor neuron (SMN) gene, the spinal muscular atrophy (SMA) disease gene, the role of the SMN protein in motor neurons and hence in the pathogenesis of SMA is still unclear. The spatial and temporal expression of SMN in neurons, particularly during development, could help in verifying the ...
Giavazzi A., Setola V., SIMONATI, Alessandro, Battaglia G.   +3 more
openaire   +3 more sources

Progranulin is expressed within motor neurons and promotes neuronal cell survival [PDF]

BMC Neuroscience, 2009
Abstract Background Progranulin is a secreted high molecular weight growth factor bearing seven and one half copies of the cysteine-rich granulin-epithelin motif. While inappropriate over-expression of the progranulin gene has been associated with many cancers, haploinsufficiency leads to atrophy of the frontotemporal
Kay Denis G, Minotti Sandra, Durham Heather D, Cao Mingju, Li Zhi, Malik Suneil, Chitramuthu Babykumari P, Baranowski David C, Ryan Cara L, Shaw Christopher A, Bennett Hugh PJ, Bateman Andrew   +11 more
openaire   +3 more sources

Central synaptopathy is the most conserved feature of motor circuit pathology across spinal muscular atrophy mouse models

iScience, 2021
Summary: Spinal muscular atrophy (SMA) is a neurodegenerative disease caused by reduced survival motor neuron (SMN) protein. Recently, SMN dysfunction has been linked to individual aspects of motor circuit pathology in a severe SMA mouse model.
Jannik M. Buettner, Josiane K. Sime Longang, Florian Gerstner, Katharina S. Apel, Beatriz Blanco-Redondo, Leonie Sowoidnich, Eva Janzen, Tobias Langenhan, Brunhilde Wirth, Christian M. Simon   +9 more
doaj   +1 more source

Decreased function of survival motor neuron protein impairs endocytic pathways. [PDF]

Proc Natl Acad Sci U S A, 2016
Dimitriadi M, Derdowski A, Kalloo G, Maginnis MS, O'Hern P, Bliska B, Sorkaç A, Nguyen KC, Cook SJ, Poulogiannis G, Atwood WJ, Hall DH, Hart AC.   +12 more
europepmc   +2 more sources

Effect of the Butyrate Prodrug Pivaloyloxymethyl Butyrate (AN9) on a Mouse Model for Spinal Muscular Atrophy. [PDF]

, 2016
Spinal muscular atrophy (SMA) is an early-onset motor neuron disease that leads to loss of muscle function. Butyrate (BA)-based compounds markedly improve the survival and motor phenotype of SMA mice.
Butchbach, Matthew E.R., Edwards, Jonathan D.   +1 more
core   +2 more sources