Cargando…

Electrophysiological Properties of Motor Neurons in a Mouse Model of Severe Spinal Muscular Atrophy: In Vitro versus In Vivo Development

We examined the electrophysiological activity of motor neurons from the mouse model of severe spinal muscular atrophy (SMA) using two different methods: whole cell patch clamp of neurons cultured from day 13 embryos; and multi-electrode recording of ventral horns in spinal cord slices from pups on p...

Descripción completa

Detalles Bibliográficos
Autores principales:	Zhang, Hongmei, Robinson, Natallia, Wu, Chiayen, Wang, Wenlan, Harrington, Melissa A.
Formato:	Texto
Lenguaje:	English
Publicado:	Public Library of Science 2010
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2908141/ https://www.ncbi.nlm.nih.gov/pubmed/20657731 http://dx.doi.org/10.1371/journal.pone.0011696

Ejemplares similares

Transcriptome Profiling of Spinal Muscular Atrophy Motor Neurons Derived from Mouse Embryonic Stem Cells
por: Maeda, Miho, et al.
Publicado: (2014)

The Alteration of Intrinsic Excitability and Synaptic Transmission in Lumbar Spinal Motor Neurons and Interneurons of Severe Spinal Muscular Atrophy Mice
por: Sun, Jianli, et al.
Publicado: (2019)

Electrophysiological biomarkers in spinal muscular atrophy: proof of concept
por: David Arnold, W, et al.
Publicado: (2014)

Spinal muscular atrophy patient-derived motor neurons exhibit hyperexcitability
por: Liu, Huisheng, et al.
Publicado: (2015)

Inherited Paediatric Motor Neuron Disorders: Beyond Spinal Muscular Atrophy
por: Teoh, Hooi Ling, et al.
Publicado: (2017)

Cerebellar structural, astrocytic, and neuronal abnormalities in the SMNΔ7 mouse model of spinal muscular atrophy
por: Cottam, Nicholas C., et al.
Publicado: (2023)

Spinal astrocyte dysfunction drives motor neuron loss in late-onset spinal muscular atrophy
por: Schmitt, Linda-Isabell, et al.
Publicado: (2023)

Motor neuron cell-nonautonomous rescue of spinal muscular atrophy phenotypes in mild and severe transgenic mouse models
por: Hua, Yimin, et al.
Publicado: (2015)

Stasimon Contributes to the Loss of Sensory Synapses and Motor Neuron Death in a Mouse Model of Spinal Muscular Atrophy
por: Simon, Christian M., et al.
Publicado: (2019)

Utility of Survival Motor Neuron ELISA for Spinal Muscular Atrophy Clinical and Preclinical Analyses
por: Kobayashi, Dione T., et al.
Publicado: (2011)

Cell cycle inhibitors protect motor neurons in an organoid model of Spinal Muscular Atrophy
por: Hor, Jin Hui, et al.
Publicado: (2018)

Modeling the phenotype of spinal muscular atrophy by the direct conversion of human fibroblasts to motor neurons
por: Zhang, Qi-Jie, et al.
Publicado: (2017)

Calpain system is altered in survival motor neuron-reduced cells from in vitro and in vivo spinal muscular atrophy models
por: de la Fuente, Sandra, et al.
Publicado: (2020)

Skeletal Muscle DNA Damage Precedes Spinal Motor Neuron DNA Damage in a Mouse Model of Spinal Muscular Atrophy (SMA)
por: Fayzullina, Saniya, et al.
Publicado: (2014)

Plastin 3 Promotes Motor Neuron Axonal Growth and Extends Survival in a Mouse Model of Spinal Muscular Atrophy
por: Alrafiah, Aziza, et al.
Publicado: (2018)

Motor unit recovery following Smn restoration in mouse models of spinal muscular atrophy
por: Comley, Laura H, et al.
Publicado: (2022)

Proteomic assessment of a cell model of spinal muscular atrophy
por: Wu, Chia-Yen, et al.
Publicado: (2011)

Sex Difference in Spinal Muscular Atrophy Patients – are Males More Vulnerable?
por: Sun, Jianli, et al.
Publicado: (2023)

Motor Neuron Gene Therapy: Lessons from Spinal Muscular Atrophy for Amyotrophic Lateral Sclerosis
por: Tosolini, Andrew P., et al.
Publicado: (2017)

Morphological Characteristics of Motor Neurons Do Not Determine Their Relative Susceptibility to Degeneration in a Mouse Model of Severe Spinal Muscular Atrophy
por: Thomson, Sophie R., et al.
Publicado: (2012)

A Comparison of Three Electrophysiological Methods for the Assessment of Disease Status in a Mild Spinal Muscular Atrophy Mouse Model
por: Li, Jia, et al.
Publicado: (2014)

Spinal muscular atrophy
por: D'Amico, Adele, et al.
Publicado: (2011)

Increasing Agrin Function Antagonizes Muscle Atrophy and Motor Impairment in Spinal Muscular Atrophy
por: Boido, Marina, et al.
Publicado: (2018)

Increased susceptibility of spinal muscular atrophy fibroblasts to camptothecin is p53-independent
por: Wu, Chia-Yen, et al.
Publicado: (2009)

Salbutamol inhibits ubiquitin-mediated survival motor neuron protein degradation in spinal muscular atrophy cells
por: Harahap, Nur Imma Fatimah, et al.
Publicado: (2015)

Investigation of New Morpholino Oligomers to Increase Survival Motor Neuron Protein Levels in Spinal Muscular Atrophy
por: Ramirez, Agnese, et al.
Publicado: (2018)

Bioenergetic status modulates motor neuron vulnerability and pathogenesis in a zebrafish model of spinal muscular atrophy
por: Boyd, Penelope J., et al.
Publicado: (2017)

Whole blood survival motor neuron protein levels correlate with severity of denervation in spinal muscular atrophy
por: Alves, Christiano R. R., et al.
Publicado: (2020)

Multifaceted roles of microRNAs: From motor neuron generation in embryos to degeneration in spinal muscular atrophy
por: Chen, Tai-Heng, et al.
Publicado: (2019)

Mid1 is associated with androgen-dependent axonal vulnerability of motor neurons in spinal and bulbar muscular atrophy
por: Ogura, Yosuke, et al.
Publicado: (2022)

Survival motor neuron protein and neurite degeneration are regulated by Gemin3 in spinal muscular atrophy motoneurons
por: Miralles, Maria P., et al.
Publicado: (2022)

Transcriptional profiling of differentially vulnerable motor neurons at pre-symptomatic stage in the Smn(2b/-) mouse model of spinal muscular atrophy
por: Murray, Lyndsay M., et al.
Publicado: (2015)

p53-dependent c-Fos expression is a marker but not executor for motor neuron death in spinal muscular atrophy mouse models
por: Buettner, Jannik M., et al.
Publicado: (2022)

Sensory Neurons Do Not Induce Motor Neuron Loss in a Human Stem Cell Model of Spinal Muscular Atrophy
por: Schwab, Andrew J., et al.
Publicado: (2014)

Clinical and Electrophysiological Changes in Pediatric Spinal Muscular Atrophy after 2 Years of Nusinersen Treatment
por: Axente, Mihaela, et al.
Publicado: (2022)

Feasibility and tolerability of multimodal peripheral electrophysiological techniques in a cohort of patients with spinal muscular atrophy
por: Ros, Leandra A.A., et al.
Publicado: (2023)

Longitudinal prospective cohort study to assess peripheral motor function with extensive electrophysiological techniques in patients with Spinal Muscular Atrophy (SMA): the SMA Motor Map protocol
por: Ros, Leandra A. A., et al.
Publicado: (2023)

Motor transmission defects with sex differences in a new mouse model of mild spinal muscular atrophy
por: Deguise, Marc-Olivier, et al.
Publicado: (2020)

The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy
por: Sleigh, James N., et al.
Publicado: (2011)

Nusinersen for spinal muscular atrophy
por: Wurster, Claudia D., et al.
Publicado: (2018)

Cannot write session to /tmp/vufind_sessions/sess_afjfatsbsikrq20s1grkc7j0j9