Results 41 to 50 of about 160,996 (342)

Apoptosis of Limb Innervating Motor Neurons and Erosion of Motor Pool Identity upon Lineage Specific Dicer Inactivation

Frontiers in Neuroscience, 2012
Diversification of mammalian spinal motor neurons into hundreds of subtypes is critical for the maintenance of body posture and coordination of complex movements.
Jun-An eChen, Jun-An eChen, Hynek eWichterle   +2 more
doaj   +1 more source

Selective vulnerability of spinal and cortical motor neuron subpopulations in delta7 SMA mice. [PDF]

PLoS ONE, 2013
Loss of the survival motor neuron gene (SMN1) is responsible for spinal muscular atrophy (SMA), the most common inherited cause of infant mortality. Even though the SMA phenotype is traditionally considered as related to spinal motor neuron loss, it ...
Paolo d'Errico, Marina Boido, Antonio Piras, Valeria Valsecchi, Elena De Amicis, Denise Locatelli, Silvia Capra, Francesco Vagni, Alessandro Vercelli, Giorgio Battaglia   +9 more
doaj   +1 more source

Viral delivery of antioxidant genes as a therapeutic strategy in experimental models of amyotrophic lateral sclerosis. [PDF]

, 2013
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with no effective treatment to date. Despite its multi-factorial aetiology, oxidative stress is hypothesized to be one of the key pathogenic mechanisms.
Azzouz, M, Higginbottom, A, Nanou, A, Ning, K, Shaw, P, Valori, CF, Wyles, M   +6 more
core   +1 more source

Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy [PDF]

, 2008
The role of heterotopic (migratory) motoneurons (HMN) in the pathogenesis of spinal muscular atrophy (SMA) is still controversial. We examined the occurrence and amount of HMN in spinal cord tissue from eight children with SMA (six with SMA-I and two ...
Barišić, Nina, Hof, Patrick R., Islam, Atiqul, Jovanov Milošević, Nataša, Krušlin, Božo, Lucassen, Paul J., Mladinov, Mihovil, Pajtak, Alen, Sertić, Jadranka, Šešo Šimić, Đurđica, Šimić, Goran   +10 more
core   +1 more source

The survival motor neuron protein in spinal muscular atrophy [PDF]

Human Molecular Genetics, 1997
The 38 kDa survival motor neuron (SMN) protein is encoded by two ubiquitously expressed genes: telomeric SMN (SMN(T)) and centromeric SMN (SMN(C)). Mutations in SMN(T), but not SMN(C), cause proximal spinal muscular atrophy (SMA), an autosomal recessive disorder that results in loss of motor neurons. SMN is found in the cytoplasm and nucleus.
D D, Coovert, T T, Le, P E, McAndrew, J, Strasswimmer, T O, Crawford, J R, Mendell, S E, Coulson, E J, Androphy, T W, Prior, A H, Burghes   +9 more
openaire   +2 more sources

The survival motor neuron protein of Schizosacharomyces pombe. Conservation of survival motor neuron interaction domains in divergent organisms. [PDF]

The Journal of biological chemistry, 2000
Spinal muscular atrophy is a common often lethal neurodegenerative disease resulting from deletions or mutations in the survival motor neuron gene (SMN). SMN is ubiquitously expressed in metazoan cells and plays a role in small nuclear ribonucleoprotein assembly and pre-mRNA splicing. Here we characterize the Schizosacharomyces pombe orthologue of SMN (
S, Paushkin, B, Charroux, L, Abel, R A, Perkinson, L, Pellizzoni, G, Dreyfuss   +5 more
openaire   +2 more sources

Drosophila Neurotrophins Reveal a Common Mechanism for Nervous System Formation [PDF]

, 2008
Neurotrophic interactions occur in Drosophila, but to date, no neurotrophic factor had been found. Neurotrophins are the main vertebrate secreted signalling molecules that link nervous system structure and function: they regulate neuronal survival ...
Aberle, Alcantara, Alicia Hidalgo, Altar, Arancio, Arenas, Arendt, Armstrong, Bangfu Zhu, Barde, Barres, Beck, Belluardo, Ben Sutcliffe, Benito-Gutierrez, Bergmann, Bergner, Blum, Booth, Bossing, Bothwell, Broihier, Buj-Bello, Burke, Campos, Cash, Chao, Chen, Chevrel, Chun-Jing Gu, Coen, Collette, Collins, Conover, Crowley, Davies, Dearborn, DeLotto, Denes, Dong, Enokido, Ernfors, Ernfors, Fischbach, Freudenthal, Gangloff, Geddes, Halfon, Halfon, Hallbö, Hashimoto, Hemmrich, Henderson, Hidalgo, Hofer, Hu, Huang, Hyman, Inamori, Jaaro, Jacob, Jacobs, Janine C Fenton, Jenny A Pennack, Keishishian, Keishishian, Kenji Mizuguchi, Kirstein, Klein, Landgraf, Landgraf, Landgraf, Landgraf, Landgraf, Learte, Lee, Levi-Montalcini, Ligoxigakis, Liu, Liu, Lu, Ma, Manjon, Manuel G Forero, Martinowich, Mattson, McCabe, Means, Memet, Michael Bate, Minichiello, Mizuguchi, Mizuguchi, Morisato, Moroz, Morris, Noordermeer, Odden, Oppenheim, Oppenheim, Ormond, Parker, Peter McQuilton, Pezet, Putnam, Rogulja-Ortmann, Rose, Roux, Rubin, Sali, Scarabelli, Schmidt, Schwartz, Schweigreiter, Sendtner, Shi, Silos-Santiago, Sink, Sink, Snider, Sofroniew, Sonnenfeld, Sossin, Stadelmann, Staniszewska, Stemerdink, Tauszig, van Kesteren, Venter, Wang, Weber, Weber, White, Wright, Xiong, Zhou, Zweifel   +136 more
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SAM68 is a physiological regulator of SMN2 splicing in spinal muscular atrophy [PDF]

, 2015
Spinal muscular atrophy (SMA) is a neurodegenerative disease caused by loss of motor neurons in patients with null mutations in the SMN1 gene. The almost identical SMN2 gene is unable to compensate for this deficiency because of the skipping of exon 7 ...
Annalisa Nobili, Antonio Musarò, Arnold, Biamonte, Bianchi, Bielli, Bielli, Bose, Branchu, Bricceno, Burnett, Bäumer, Cai, Chen, Cho, Claudio Sette, Ehrmann, Feng, Fu, Galarneau, Hofmann, Hofmann, Hua, Huot, Iascone, Iijima, Kashima, Kashima, Laura Pelosi, Le, Li, Ling, Lorson, Lukong, Mammucari, Marcello D’Amelio, Maria Blaire Bustamante, Maria Grazia Berardinelli, Mende, Nurputra, Paronetto, Paronetto, Pedrotti, Pedrotti, Richard, Ruggiu, Shababi, Singh, Vittoria Pagliarini, Wahl, West, Zhang   +51 more
core   +2 more sources

Restoration of SMN in Schwann cells reverses myelination defects and improves neuromuscular function in spinal muscular atrophy [PDF]

, 2016
Spinal muscular atrophy (SMA) is a neuromuscular disease caused by low levels of SMN protein, primarily affecting lower motor neurons. Recent evidence from SMA and related conditions suggests that glial cells can influence disease severity.
Brophy, Peter J., Gillingwater, Thomas H., Groen, Ewout J.N., Hunter, Gillian, Jones, Ross A., Lane, Fiona M., Powis, Rachael A., Sherman, Diane L., Shorrock, Hannah K., Zheng, Yinan   +9 more
core   +7 more sources

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