Results 51 to 60 of about 159,037 (298)

Reduced VGLUT2 expression increases motor neuron viability in Sod1G93A mice

Neurobiology of Disease, 2010
Glutamate-induced excitotoxicity has been suggested to influence pathogenesis in amyotrophic lateral sclerosis (ALS). Vesicular glutamate transporters (VGLUTs) are responsible for transport of glutamate into synaptic vesicles.
Hanna Wootz, Anders Enjin, Åsa Wallén-Mackenzie, Dan Lindholm, Klas Kullander   +4 more
doaj   +1 more source

Efficient and rapid conversion of human astrocytes and ALS mouse model spinal cord astrocytes into motor neuron-like cells by defined small molecules

Military Medical Research, 2020
Background Motor neuron degeneration or loss in the spinal cord is the characteristic phenotype of motor neuron diseases or spinal cord injuries. Being proliferative and located near neurons, astrocytes are considered ideal cell sources for regenerating ...
An-Dong Zhao, Hua Qin, Meng-Li Sun, Kui Ma, Xiao-Bing Fu   +4 more
doaj   +1 more source

Vascular regression precedes motor neuron loss in the FUS (1-359) ALS mouse model

Disease Models & Mechanisms, 2019
Amyotrophic lateral sclerosis (ALS) presents a poorly understood pathogenesis. Evidence from patients and mutant SOD1 mouse models suggests vascular damage may precede or aggravate motor dysfunction in ALS.
Martin Crivello, Marion C. Hogg, Elisabeth Jirström, Luise Halang, Ina Woods, Megan Rayner, Karen S. Coughlan, Sebastian A. Lewandowski, Jochen H. M. Prehn   +8 more
doaj   +1 more source

WDR79/TCAB1 plays a conserved role in the control of locomotion and ameliorates phenotypic defects in SMA models [PDF]

, 2017
SMN (Survival Motor Neuron) deficiency is the predominant cause of spinal muscular atrophy (SMA), a severe neurodegenerative disorder that can lead to progressive paralysis and death.
Bavasso, Francesca, Cacchione, Stefano, DI GIORGIO, MARIA LAURA, Esposito, Alessandro, Feiguin, Fabian, Gallotta, Ivan, Maccallini, Paolo, Mccabe, Brian D., Micheli, Emanuela, Raffa, GRAZIA DANIELA, Schiavi, Elia Di, Verni', Fiammetta   +11 more
core   +1 more source

Motor alterations induced by chronic 4-aminopyridine infusion in the spinal cord in vivo: role of glutamate and GABA receptors

Frontiers in Neuroscience, 2016
Motor neuron degeneration is the pathological hallmark of motor neuron diseases, a group of neurodegenerative disorders clinically manifested as muscle fasciculations and hyperreflexia, followed by paralysis, respiratory failure and death. Ample evidence
Rafael eLazo-Gómez, Ricardo eTapia
doaj   +1 more source

CRISPR-Cas9 screens in human cells and primary neurons identify modifiers of C9ORF72 dipeptide-repeat-protein toxicity. [PDF]

, 2018
Hexanucleotide-repeat expansions in the C9ORF72 gene are the most common cause of amyotrophic lateral sclerosis and frontotemporal dementia (c9ALS/FTD).
A Berson, A Jovičić, AA Dominguez, Aaron D. Gitler, AC Elden, AD Gitler, AD Gitler, AD Gitler, AE Renton, AM Jablonski, Amy Li, Ana Jovičić, AS Ng, BD Freibaum, C Sidrauski, C Sidrauski, C. Kimberly Tsui, D Ron, David W. Morgens, DW Morgens, DW Morgens, E Yeger-Lotem, EM Lynes, EY Son, FB Gao, Gregor Bieri, H Koike-Yusa, HJ Kim, HJ Kim, I Kwon, IR Mackenzie, IR Mackenzie, J Couthouis, J Joung, James Ousey, JC Christianson, JG Wideman, JP Taylor, Julien Couthouis, Justin K. Ichida, K Kanekura, K Mori, K Zhang, KH Lee, KM Green, KY Shi, L Ellgaard, L Huang, LA Becker, LA Gilbert, LA Gilbert, M Armakola, M DeJesus-Hernandez, M Neumann, M Prudencio, MA Horlbeck, Marc Tessier-Lavigne, MC Jonikas, MH Schludi, MI Love, Michael C. Bassik, Michael S. Haney, NF Liachko, Nicholas J. Kramer, Nicholas T. Hertz, O Shalem, OM Peters, PE Ash, Q Zhou, R Dafinca, R Lopez-Gonzalez, R Seijffers, RJ Platt, RM Deans, Rosanna Ma, S Boeynaems, S Boeynaems, S Ju, S Konermann, S Mizielinska, S Treusch, SC Ling, SJ Zhang, T Wang, T Westergard, T Zu, TF Gendron, W Cheng, X Wen, Y Lin, Y Zhou, Yingxiao Shi, YJ Zhang, YS Davidson, Z Shevtsova, Z Sun   +95 more
core   +1 more source

Is Good Housekeeping the Key to Motor Neuron Survival? [PDF]

Cell, 2008
Spinal muscular atrophy (SMA) is caused by a drastic reduction in the ubiquitously expressed SMN protein, which is critical for the correct assembly of the snRNP complexes required for RNA splicing. However, it is unclear why loss of SMN and altered snRNP assembly only seem to affect motor neurons. Reporting in this issue, Zhang et al. (2008) challenge
Talbot, K, Davies, K
openaire   +3 more sources

Dysregulation of microRNA metabolism in motor neuron diseases: Novel biomarkers and potential therapeutics

Non-coding RNA Research, 2019
In the last 15 years, several classes of small regulatory RNAs have been identified, uncovering the widespread impact of non-coding elements in the human genome on cell homeostasis and human diseases.
Elisa De Paola, Veronica Verdile, Maria Paola Paronetto   +2 more
doaj   +1 more source

Inhibition of apoptosis blocks human motor neuron cell death in a stem cell model of spinal muscular atrophy. [PDF]

PLoS ONE, 2012
Spinal muscular atrophy (SMA) is a genetic disorder caused by a deletion of the survival motor neuron 1 gene leading to motor neuron loss, muscle atrophy, paralysis, and death.
Dhruv Sareen, Allison D Ebert, Brittany M Heins, Jered V McGivern, Loren Ornelas, Clive N Svendsen   +5 more
doaj   +1 more source

Organoids in pediatric cancer research

FEBS Letters, EarlyView.
Organoid technology has revolutionized cancer research, yet its application in pediatric oncology remains limited. Recent advances have enabled the development of pediatric tumor organoids, offering new insights into disease biology, treatment response, and interactions with the tumor microenvironment.
Carla Ríos Arceo, Jarno Drost
wiley   +1 more source