Characterization of behavioral and neuromuscular junction phenotypes in a novel allelic series of SMA mouse models - Wikidata - awgldk - TriplyDB

Characterization of behavioral and neuromuscular junction phenotypes in a novel allelic series of SMA mouse models

Characterization of behavioral and neuromuscular junction phenotypes in a novel allelic series of SMA mouse models

http://www.wikidata.org/entity/Q36283365

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A comparison of three electrophysiological methods for the assessment of disease status in a mild spinal muscular atrophy mouse model Pharmacokinetics, pharmacodynamics, and efficacy of a small-molecule SMN2 splicing modifier in mouse models of spinal muscular atrophy Pharmacology of a central nervous system delivered 2'-O-methoxyethyl-modified survival of motor neuron splicing oligonucleotide in mice and nonhuman primates.Assays for the identification and prioritization of drug candidates for spinal muscular atrophy SMN2 splice modulators enhance U1-pre-mRNA association and rescue SMA mice.Motor neuron disease. SMN2 splicing modifiers improve motor function and longevity in mice with spinal muscular atrophy.Spinal muscular atrophy: journeying from bench to bedside SMN Protein Can Be Reliably Measured in Whole Blood with an Electrochemiluminescence (ECL) Immunoassay: Implications for Clinical Trials SMN deficiency disrupts gastrointestinal and enteric nervous system function in mice Small Molecules in Development for the Treatment of Spinal Muscular Atrophy.Activin Receptor Type IIB Inhibition Improves Muscle Phenotype and Function in a Mouse Model of Spinal Muscular Atrophy Systemic, postsymptomatic antisense oligonucleotide rescues motor unit maturation delay in a new mouse model for type II/III spinal muscular atrophy.Severe impairment of male reproductive organ development in a low SMN expressing mouse model of spinal muscular atrophy A novel human-specific splice isoform alters the critical C-terminus of Survival Motor Neuron protein.Spinal muscular atrophy: an update on therapeutic progress More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases.Cardiac pathology in spinal muscular atrophy: a systematic review.TIA1 is a gender-specific disease modifier of a mild mouse model of spinal muscular atrophy.A Stem Cell Model of the Motor Circuit Uncouples Motor Neuron Death from Hyperexcitability Induced by SMN Deficiency.Gender-Specific Amelioration of SMA Phenotype upon Disruption of a Deep Intronic Structure by an Oligonucleotide.Optimizing mouse models of neurodegenerative disorders: are therapeutics in sight?

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Characterization of behavioral and neuromuscular junction phenotypes in a novel allelic series of SMA mouse models

http://www.wikidata.org/entity/Q36283365

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2012 nî lūn-bûn

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Human Molecular Genetics

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Characterization of behavioral ...... lic series of SMA mouse models

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Afshin Ghavami

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Bassem El-Khodor

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Cathleen Lutz

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Chien-Ping Ko

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Corissa McEwen

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Crystal Davis

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David D McKemy

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Dione Kobayashi

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Jose Beltran

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Synaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophy

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Arrhythmia and cardiac defects are a feature of spinal muscular atrophy model mice.

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10.1093/HMG/DDS285

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20

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21

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Laurent Bogdanik

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2012-07-16T00:00:00Z

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22802075

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3459466

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