Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept. - Wikidata - wikimedia - TriplyDB

Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.

Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.

http://www.wikidata.org/entity/Q37556531

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A large animal model of spinal muscular atrophy and correction of phenotype.A comparison of three electrophysiological methods for the assessment of disease status in a mild spinal muscular atrophy mouse model Spinal muscular atrophy: diagnosis and management in a new therapeutic era Spinal muscular atrophy: from tissue specificity to therapeutic strategies Improving single injection CSF delivery of AAV9-mediated gene therapy for SMA: a dose-response study in mice and nonhuman primates.Plastin 3 Expression Does Not Modify Spinal Muscular Atrophy Severity in the ∆7 SMA Mouse Defective membrane fusion and repair in Anoctamin5-deficient muscular dystrophy.SMN expression is required in motor neurons to rescue electrophysiological deficits in the SMNΔ7 mouse model of SMA.Normalization of Patient-Identified Plasma Biomarkers in SMNΔ7 Mice following Postnatal SMN Restoration.Systemic, postsymptomatic antisense oligonucleotide rescues motor unit maturation delay in a new mouse model for type II/III spinal muscular atrophy.AAV1.NT-3 gene therapy attenuates spontaneous autoimmune peripheral polyneuropathy.The neuromuscular impact of symptomatic SMN restoration in a mouse model of spinal muscular atrophy.Single and modeled multifrequency electrical impedance myography parameters and their relationship to force production in the ALS SOD1G93A mouse How the discovery of ISS-N1 led to the first medical therapy for spinal muscular atrophy Antisense Oligonucleotides: Translation from Mouse Models to Human Neurodegenerative Diseases.Electrophysiological Motor Unit Number Estimation (MUNE) Measuring Compound Muscle Action Potential (CMAP) in Mouse Hindlimb Muscles.Electrical impedance myography detects age-related muscle change in mice.In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration Evaluation of potential effects of Plastin 3 overexpression and low-dose SMN-antisense oligonucleotides on putative biomarkers in spinal muscular atrophy mice

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P2860

Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.

http://www.wikidata.org/entity/Q37556531

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article scientifique

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artigo científico

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bilimsel makale

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scientific article published on January 2014

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vedecký článok

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vetenskaplig artikel

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videnskabelig artikel

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vědecký článek

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name

Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.

@en

Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.

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Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.

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Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.

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prefLabel

Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.

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Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.

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Annals of clinical and translational neurology

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Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept

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Brian K Kaspar

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Chitra C Iyer

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John T Kissel

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Kathrin Meyer

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Leah Schmelzer

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Paul N Porensky

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Sandra Duque

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Stephen J Kolb

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Vicki L McGovern

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W David Arnold

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P2860

Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy

Spinal muscular atrophy: development and implementation of potential treatments

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

Regulation of SMN protein stability

Smn, the spinal muscular atrophy-determining gene product, modulates axon growth and localization of beta-actin mRNA in growth cones of motoneurons

Identification and characterization of a spinal muscular atrophy-determining gene

A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy

Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy.

Dorsal caudal tail and sciatic motor nerve conduction studies in adult mice: technical aspects and normative data.

Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?

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34-44

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scholarly article

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10.1002/ACN3.23

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1

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2014-01-01T00:00:00Z

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260131986

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24511555

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electrophysiology

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3914317

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