Results 21 to 30 of about 73,691 (271)

Mutant glycyl-tRNA synthetase (Gars) ameliorates SOD1G93A motor neuron degeneration phenotype but has little affect on Loa dynein heavy chain mutant mice [PDF]

, 2009
Background: In humans, mutations in the enzyme glycyl-tRNA synthetase (GARS) cause motor and sensory axon loss in the peripheral nervous system, and clinical phenotypes ranging from Charcot-Marie-Tooth neuropathy to a severe infantile form of spinal ...
Hazel P Williams, Greensmith Linda, Francesca Achilli, Ruth Chia, Elizabeth M. C. Fisher, Hazel P. Williams, Fisher Elizabeth M. C., Achilli, F., Chia, R, Bryson, J.B., Bros-Facer, V., Greensmith, L., Bros-Facer, V, Banks, G.T., Bryson, JB, Banks, GT, Fisher, EMC, Bros-Facer Virginie, Linda Greensmith, Fisher, E.M.C., Banks Gareth T., Chia, R., Gareth T Banks, Williams, HP, Greensmith, L, Williams Hazel P., Elizabeth M C Fisher, J Barney Bryson, Gareth T. Banks, Virginie Bros-Facer, Achilli Francesca, Williams, H.P., Bryson J. Barney, Chia Ruth, J. Barney Bryson, Achilli, F   +35 more
core   +1 more source

Effects of survival motor neuron protein on germ cell development in mouse and human [PDF]

, 2022
Survival motor neuron (SMN) is ubiquitously expressed in many cell types and its encoding gene, survival motor neuron 1 gene (SMN1), is highly conserved in various species.
Peng, M, Cho, HC, Hsieh-Li, HM, Sung, LY, Xu, J, Liu, JL, Lu, CH, Hsu, J, Chang, WF   +8 more
core   +1 more source

Altered Metabolic Profiles Associate with Toxicity in SOD1G93A Astrocyte-Neuron Co-Cultures

Scientific Reports, 2017
Non-cell autonomous processes involving astrocytes have been shown to contribute to motor neuron degeneration in amyotrophic lateral sclerosis. Mutant superoxide dismutase 1 (SOD1G93A) expression in astrocytes is selectively toxic to motor neurons in co ...
Gabriel N. Valbuena, Massimo Tortarolo, Caterina Bendotti, Lavinia Cantoni, Hector C. Keun   +4 more
doaj   +1 more source

Decreased microRNA levels lead to deleterious increases in neuronal M2 muscarinic receptors in Spinal Muscular Atrophy models

eLife, 2017
Spinal Muscular Atrophy (SMA) is caused by diminished Survival of Motor Neuron (SMN) protein, leading to neuromuscular junction (NMJ) dysfunction and spinal motor neuron (MN) loss.
Patrick J O'Hern, Inês do Carmo G. Gonçalves, Johanna Brecht, Eduardo Javier López Soto, Jonah Simon, Natalie Chapkis, Diane Lipscombe, Min Jeong Kye, Anne C Hart   +8 more
doaj   +1 more source

The Peroxisome Proliferator-activated Receptor γ (PPARγ) Controls Natural Protective Mechanisms against Lipid Peroxidation in Amyotrophic Lateral Sclerosis [PDF]

, 2012
Recent evidence highlights the peroxisome proliferator-activated receptors (PPARs) as critical neuroprotective factors in several neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). To gain new mechanistic insights into the role of
Maggi, Adriana, L. Brambilla, Benedusi, Valeria, Brambilla, Liliana, A. Maggi, V. Benedusi, Martorana, Francesca, F. Martorana, Rossi, Daniela   +8 more
core   +1 more source

The distribution and function of GDE2, a regulator of spinal motor neuron survival, are disrupted in Amyotrophic Lateral Sclerosis

Acta Neuropathologica Communications, 2022
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that affects the viability of upper and lower motor neurons. Current options for treatment are limited, necessitating deeper understanding of the mechanisms underlying ALS ...
Anna Westerhaus, Thea Joseph, Alison J. Meyers, Yura Jang, Chan Hyun Na, Clinton Cave, Shanthini Sockanathan   +6 more
doaj   +1 more source

Gene profiling of identified neurons to dissect molecular mechanisms involved in spinal reflex assembly [PDF]

, 2010
The central question during my PhD studies was to understand the molecular mechanisms and genetic cascades controlling the sequential specification of distinct classes of dorsal root ganglia (DRG) sensory neurons, with a particular focus on genes ...
Friese, Andreas
core   +1 more source

Prolonged minocycline treatment impairs motor neuronal survival and glial function in organotypic rat spinal cord cultures. [PDF]

PLoS ONE, 2013
BACKGROUND: Minocycline, a second-generation tetracycline antibiotic, exhibits anti-inflammatory and neuroprotective effects in various experimental models of neurological diseases, such as stroke, Alzheimer's disease, amyotrophic lateral sclerosis and ...
Josephine Pinkernelle, Hisham Fansa, Uwe Ebmeyer, Gerburg Keilhoff   +3 more
doaj   +1 more source

Binding of the heterogeneous ribonucleoprotein K (hnRNP K) to the Epstein-Barr virus nuclear antigen 2 (EBNA2) enhances viral LMP2A expression. [PDF]

, 2012
The Epstein-Barr Virus (EBV) -encoded EBNA2 protein, which is essential for the in vitro transformation of B-lymphocytes, interferes with cellular processes by binding to proteins via conserved sequence motifs.
Stephanie Barth, Neuenkirchen, N., Alfredo Mamiani (146650), Grässer, Friedrich A., Ostareck-Lederer, Antje, Leonhardt, Heinrich, Nils Neuenkirchen (146673), Fischer, Utz, Bodo Moritz, Friedrich A Grässer, Wen Deng, Masouris, Ilias, Barth, Stephanie, Fischer, U., Hennard, C., Christian Cassel, Neuenkirchen, Nils, Utz Fischer (146680), Utz Fischer, Ute Stober-Grässer (146645), Elfriede Noessner (146689), Noessner, Elfriede, Henrik Gross (146622), Bodo Moritz (146655), Dirk Ostareck, Mamiani, Alfredo, Stephanie Barth (145387), Nößner, E., Alfredo Mamiani, Ostareck, Dirk, Christine Hennard (146627), Christian Cassel (146638), Moritz, Bodo, Antje Ostareck-Lederer, Elfriede Noessner, Deng, Wen, Gross, H., Christine Hennard, Ostareck, Dirk H., Antje Ostareck-Lederer (146668), Dirk Ostareck (146662), Heinrich Leonhardt (18589), Henrik Gross, Stober-Grässer, U., Masouris, I., Friedrich A. Grässer (145412), Kremmer, E., Wen Deng (123626), Grässer, F.A., Kremmer, Elisabeth, Cassel, C., Deng, W., Ilias Masouris (146632), Ilias Masouris, Ostareck-Lederer, A., Mamiani, A., Heinrich Leonhardt, Gross, Henrik, Moritz, B., Nils Neuenkirchen, Barth, S., Ostareck, D., Cassel, Christian, Stober-Grässer, Ute, Elisabeth Kremmer (17351), Ute Stober-Grässer, Elisabeth Kremmer, Leonhardt, H., Hennard, Christine   +68 more
core   +1 more source

ZPR1-Dependent Neurodegeneration Is Mediated by the JNK Signaling Pathway

Journal of Experimental Neuroscience, 2019
The zinc finger protein ZPR1 deficiency causes neurodegeneration and results in a mild spinal muscular atrophy (SMA)-like disease in mice with reduced Zpr1 gene dosage. Mutation of the survival motor neuron 1 ( SMN1 ) gene causes SMA.
Xiaoting Jiang, Annapoorna Kannan, Laxman Gangwani   +2 more
doaj   +1 more source