Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons - Wikidata - awgldk - TriplyDB

Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons

Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons

http://www.wikidata.org/entity/Q35006040

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Neuromuscular Junctions as Key Contributors and Therapeutic Targets in Spinal Muscular Atrophy Selective Neuromuscular Denervation in Taiwanese Severe SMA Mouse Can Be Reversed by Morpholino Antisense Oligonucleotides Muscle-derived extracellular signal-regulated kinases 1 and 2 are required for the maintenance of adult myofibers and their neuromuscular junctions.Non-aggregating tau phosphorylation by cyclin-dependent kinase 5 contributes to motor neuron degeneration in spinal muscular atrophy.SMN deficiency disrupts gastrointestinal and enteric nervous system function in mice SMN expression is required in motor neurons to rescue electrophysiological deficits in the SMNΔ7 mouse model of SMA.Low levels of Survival Motor Neuron protein are sufficient for normal muscle function in the SMNΔ7 mouse model of SMA.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.Transcriptional enhancement of Smn levels in motoneurons is crucial for proper axon morphology in zebrafish.Defective Acetylcholine Receptor Subunit Switch Precedes Atrophy of Slow-Twitch Skeletal Muscle Fibers Lacking ERK1/2 Kinases in Soleus Muscle.Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.Restoration of SMN in Schwann cells reverses myelination defects and improves neuromuscular function in spinal muscular atrophy.SMN deficiency does not induce oxidative stress in SMA iPSC-derived astrocytes or motor neurons.Astrocytes influence the severity of spinal muscular atrophy.SMA-causing missense mutations in survival motor neuron (Smn) display a wide range of phenotypes when modeled in Drosophila.Decreased Motor Neuron Support by SMA Astrocytes due to Diminished MCP1 Secretion.The Antisense Transcript SMN-AS1 Regulates SMN Expression and Is a Novel Therapeutic Target for Spinal Muscular Atrophy.Increasing Agrin Function Antagonizes Muscle Atrophy and Motor Impairment in Spinal Muscular Atrophy.

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Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons

http://www.wikidata.org/entity/Q35006040

description

2013 nî lūn-bûn

@nan

2013 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած

@hyw

2013 թվականի սեպտեմբերին հրատարակված գիտական հոդված

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2013年の論文

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2013年論文

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2013年論文

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2013年論文

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2013年論文

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2013年論文

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2013年论文

@wuu

name

Improvement of neuromuscular s ...... ively rescued in motor neurons

@ast

Improvement of neuromuscular s ...... ively rescued in motor neurons

@en

type

label

Improvement of neuromuscular s ...... ively rescued in motor neurons

@ast

Improvement of neuromuscular s ...... ively rescued in motor neurons

@en

prefLabel

Improvement of neuromuscular s ...... ively rescued in motor neurons

@ast

Improvement of neuromuscular s ...... ively rescued in motor neurons

@en

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Improvement of neuromuscular s ...... ively rescued in motor neurons

@en

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Bonnie Seaberg

http://www.w3.org/2001/XMLSchema#string

Charlotte J Sumner

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Lingling Kong

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Mendell Rimer

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Tara L Martinez

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P2860

Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2−ΔΔCT Method

Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy.

Requirement for the homeobox gene Hb9 in the consolidation of motor neuron identity

Patterning of muscle acetylcholine receptor gene expression in the absence of motor innervation

CNS-targeted gene therapy improves survival and motor function in a mouse model of spinal muscular atrophy.

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

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

Mouse survival motor neuron alleles that mimic SMN2 splicing and are inducible rescue embryonic lethality early in development but not late

SMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminals

Reduced survival of motor neuron (SMN) protein in motor neuronal progenitors functions cell autonomously to cause spinal muscular atrophy in model mice expressing the human centromeric (SMN2) gene

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9

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Ximena Paez-Colasante

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2013-09-23T00:00:00Z

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257302755

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24086650

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spinal muscular atrophy

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3781079

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