The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy
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Neuroplasticity and Repair in Rodent Neurotoxic Models of Spinal Motoneuron DiseaseUBA1: At the Crossroads of Ubiquitin Homeostasis and NeurodegenerationGeneration of Functional Neuromuscular Junctions from Human Pluripotent Stem Cell Lines.Chondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophyA comparison of three electrophysiological methods for the assessment of disease status in a mild spinal muscular atrophy mouse modelIdentification of a Peptide for Systemic Brain Delivery of a Morpholino Oligonucleotide in Mouse Models of Spinal Muscular AtrophyMorphological analysis of neuromuscular junction development and degeneration in rodent lumbrical muscles.Invertebrate models of spinal muscular atrophy: insights into mechanisms and potential therapeutics.GEMINs: potential therapeutic targets for spinal muscular atrophy?Neuromuscular junction maturation defects precede impaired lower motor neuron connectivity in Charcot-Marie-Tooth type 2D miceSpinal Muscular Atrophy Therapeutics: Where do we Stand?Morphological characteristics of motor neurons do not determine their relative susceptibility to degeneration in a mouse model of severe spinal muscular atrophyDisease mechanisms and therapeutic approaches in spinal muscular atrophy.Different Stability and Proteasome-Mediated Degradation Rate of SMN Protein Isoforms.Dominant, toxic gain-of-function mutations in gars lead to non-cell autonomous neuropathology.Spinal muscular atrophy: the role of SMN in axonal mRNA regulation.Transcriptional profiling of differentially vulnerable motor neurons at pre-symptomatic stage in the Smn (2b/-) mouse model of spinal muscular atrophy.Survival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophyDecay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons.A functional motor unit in the culture dish: co-culture of spinal cord explants and muscle cells.Mechanistic principles of antisense targets for the treatment of spinal muscular atrophyα-COP binding to the survival motor neuron protein SMN is required for neuronal process outgrowthBehavioral and electrophysiological outcomes of tissue-specific Smn knockdown in Drosophila melanogaster.Spinal Muscular Atrophy Patient iPSC-Derived Motor Neurons Have Reduced Expression of Proteins Important in Neuronal Development.Chronic Treatment with the AMPK Agonist AICAR Prevents Skeletal Muscle Pathology but Fails to Improve Clinical Outcome in a Mouse Model of Severe Spinal Muscular Atrophy.The DcpS inhibitor RG3039 improves motor function in SMA mice.The Power of Human Protective Modifiers: PLS3 and CORO1C Unravel Impaired Endocytosis in Spinal Muscular Atrophy and Rescue SMA PhenotypeSpinal muscular atrophy: an update on therapeutic progressSMN-targeted therapeutics for spinal muscular atrophy: are we SMArt enough yet?Overexpression of survival motor neuron improves neuromuscular function and motor neuron survival in mutant SOD1 mice.Spinal muscular atrophy astrocytes exhibit abnormal calcium regulation and reduced growth factor productionGem depletion: amyotrophic lateral sclerosis and spinal muscular atrophy crossover.Advances in understanding the role of disease-associated proteins in spinal muscular atrophy.Discovery of a Small Molecule Probe That Post-Translationally Stabilizes the Survival Motor Neuron Protein for the Treatment of Spinal Muscular Atrophy.Modeling the early phenotype at the neuromuscular junction of spinal muscular atrophy using patient-derived iPSCs.The SMA Trust: the role of a disease-focused research charity in developing treatments for SMA.TIA1 is a gender-specific disease modifier of a mild mouse model of spinal muscular atrophy.Neuropilin 1 sequestration by neuropathogenic mutant glycyl-tRNA synthetase is permissive to vascular homeostasisPlastin 3 ameliorates spinal muscular atrophy via delayed axon pruning and improves neuromuscular junction functionality.Diabetic Ketoacidosis in an Adult Patient With Spinal Muscular Atrophy Type II: Further Evidence of Extraneural Pathology Due to Survival Motor Neuron 1 Mutation?
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P2860
The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy
description
2011 nî lūn-bûn
@nan
2011 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
name
The contribution of mouse mode ...... sis of spinal muscular atrophy
@ast
The contribution of mouse mode ...... sis of spinal muscular atrophy
@en
The contribution of mouse mode ...... sis of spinal muscular atrophy
@nl
type
label
The contribution of mouse mode ...... sis of spinal muscular atrophy
@ast
The contribution of mouse mode ...... sis of spinal muscular atrophy
@en
The contribution of mouse mode ...... sis of spinal muscular atrophy
@nl
prefLabel
The contribution of mouse mode ...... sis of spinal muscular atrophy
@ast
The contribution of mouse mode ...... sis of spinal muscular atrophy
@en
The contribution of mouse mode ...... sis of spinal muscular atrophy
@nl
P2860
P50
P921
P356
P1476
The contribution of mouse mode ...... sis of spinal muscular atrophy
@en
P2860
P304
P356
10.1242/DMM.007245
P407
P577
2011-07-01T00:00:00Z