Results 11 to 20 of about 160,996 (342)

Survival of motor neuron 1 telomeric (SMN1; SMN); survival of motor neuron 2 centromeric (SMN2; SMNC) [PDF]

Science-Business eXchange, 2009

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Survival motor neuron protein deficiency alters microglia reactivity. [PDF]

Glia, 2022
AbstractSurvival motor neuron (SMN) protein deficiency results in loss of alpha motor neurons and subsequent muscle atrophy in patients with spinal muscular atrophy (SMA). Reactive microglia have been reported in SMA mice and depleting microglia rescues the number of proprioceptive synapses, suggesting a role in SMA pathology.
Khayrullina G, Alipio-Gloria ZA, Deguise MO, Gagnon S, Chehade L, Stinson M, Belous N, Bergman EM, Lischka FW, Rotty J, Dalgard CL, Kothary R, Johnson KA, Burnett BG.   +13 more
europepmc   +3 more sources

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.
Kelvin K. Hui, Nicole Liadis, Jennifer Robertson, Anish Kanungo, Jeffrey T. Henderson   +4 more
openalex   +5 more sources

Diverse role of survival motor neuron protein. [PDF]

Biochim Biophys Acta Gene Regul Mech, 2017
The multifunctional Survival Motor Neuron (SMN) protein is required for the survival of all organisms of the animal kingdom. SMN impacts various aspects of RNA metabolism through the formation and/or interaction with ribonucleoprotein (RNP) complexes. SMN regulates biogenesis of small nuclear RNPs, small nucleolar RNPs, small Cajal body-associated RNPs,
Singh RN, Howell MD, Ottesen EW, Singh NN.   +3 more
europepmc   +5 more sources

Phrenic motor neuron survival below cervical spinal cord hemisection

Experimental Neurology, 2021
Cervical spinal cord injury (cSCI) severs bulbospinal projections to respiratory motor neurons, paralyzing respiratory muscles below the injury. C2 spinal hemisection (C2Hx) is a model of cSCI often used to study spontaneous and induced plasticity and breathing recovery post-injury.
Latoya L. Allen, Nicole L. Nichols, A. Zachary, Anna T. Emery, Marissa C. Ciesla, Juliet V. Santiago, Ashley Holland, Gordon S. Mitchell, Elisa J. Gonzalez‐Rothi   +8 more
openalex   +4 more sources

Survival motor neuron protein modulates neuron-specific apoptosis [PDF]

Proceedings of the National Academy of Sciences, 2000
Spinal muscular atrophy (SMA) is attributed to mutations in the SMN1 gene, leading to loss of spinal cord motor neurons. The neurotropic Sindbis virus vector system was used to investigate a role for the survival motor neuron (SMN) protein in regulating neuronal apoptosis.
D A, Kerr, J P, Nery, R J, Traystman, B N, Chau, J M, Hardwick   +4 more
openaire   +2 more sources

Birth of spinal muscular atrophy unaffected baby from genetically at-risk parents following a pre-implantation genetic screening: A case report

International Journal of Reproductive BioMedicine, 2022
Background: Spinal muscular atrophy (SMA) is characterized by the homozygous deletion of the survival motor neuron-1 gene. Pre-implantation genetic testing for monogenic diseases through in-vitrofertilization program was developed to provide a reliable ...
Arie Adrainus Polim, Nining Handayani, Dian Kesumapramudya Nurputra, Anggia Melanie Lubis, Batara Sirait, Dennis Jakobus, Arief Boediono, Ivan Sini   +7 more
doaj   +1 more source

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

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