Cardiac defects contribute to the pathology of spinal muscular atrophy models - Wikidata - awgldk - TriplyDB

Cardiac defects contribute to the pathology of spinal muscular atrophy models

Cardiac defects contribute to the pathology of spinal muscular atrophy models

http://www.wikidata.org/entity/Q28290032

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Spinal muscular atrophy The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy Is spinal muscular atrophy a disease of the motor neurons only: pathogenesis and therapeutic implications?Prolactin increases SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway.SMN in spinal muscular atrophy and snRNP biogenesis Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy.The DcpS inhibitor RG3039 improves survival, function and motor unit pathologies in two SMA mouse models.Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation.PTEN depletion decreases disease severity and modestly prolongs survival in a mouse model of spinal muscular atrophy.Protective effects of butyrate-based compounds on a mouse model for spinal muscular atrophy.Rescue of a Mouse Model of Spinal Muscular Atrophy With Respiratory Distress Type 1 by AAV9-IGHMBP2 Is Dose Dependent.Somatic Therapy of a Mouse SMA Model with a U7 snRNA Gene Correcting SMN2 Splicing Emerging therapies and challenges in spinal muscular atrophy Immune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy mice The effect of diet on the protective action of D156844 observed in spinal muscular atrophy mice.Mouse survival motor neuron alleles that mimic SMN2 splicing and are inducible rescue embryonic lethality early in development but not late A short antisense oligonucleotide ameliorates symptoms of severe mouse models of spinal muscular atrophy ROCK inhibition as a therapy for spinal muscular atrophy: understanding the repercussions on multiple cellular targets Temporal requirement for high SMN expression in SMA mice Systemic gene delivery in large species for targeting spinal cord, brain, and peripheral tissues for pediatric disorders New therapeutic approaches to spinal muscular atrophy.A single administration of morpholino antisense oligomer rescues spinal muscular atrophy in mouse SMN-inducing compounds for the treatment of spinal muscular atrophy.Intramuscular scAAV9-SMN injection mediates widespread gene delivery to the spinal cord and decreases disease severity in SMA mice.Antisense oligonucleotides for the treatment of spinal muscular atrophy.Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy.ISS-N1 makes the First FDA-approved Drug for Spinal Muscular Atrophy.Increased IGF-1 in muscle modulates the phenotype of severe SMA mice Spinal muscular atrophy: from tissue specificity to therapeutic strategies Motor neuron cell-nonautonomous rescue of spinal muscular atrophy phenotypes in mild and severe transgenic mouse models Glucose metabolism and pancreatic defects in spinal muscular atrophy Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons Peripheral SMN restoration is essential for long-term rescue of a severe spinal muscular atrophy mouse model.Non-aggregating tau phosphorylation by cyclin-dependent kinase 5 contributes to motor neuron degeneration in spinal muscular atrophy.Direct central nervous system delivery provides enhanced protection following vector mediated gene replacement in a severe model of spinal muscular atrophy.Disease mechanisms and therapeutic approaches in spinal muscular atrophy.Cardiac involvement in patients with spinal muscular atrophies.Decreasing disease severity in symptomatic, Smn(-/-);SMN2(+/+), spinal muscular atrophy mice following scAAV9-SMN delivery Partial restoration of cardio-vascular defects in a rescued severe model of spinal muscular atrophy.SMN deficiency disrupts gastrointestinal and enteric nervous system function in mice

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P2860

Cardiac defects contribute to the pathology of spinal muscular atrophy models

http://www.wikidata.org/entity/Q28290032

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2010 nî lūn-bûn

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2010 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած

@hyw

2010 թվականի հոտեմբերին հրատարակված գիտական հոդված

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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name

Cardiac defects contribute to the pathology of spinal muscular atrophy models

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Cardiac defects contribute to the pathology of spinal muscular atrophy models

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Cardiac defects contribute to the pathology of spinal muscular atrophy models

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type

label

Cardiac defects contribute to the pathology of spinal muscular atrophy models

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Cardiac defects contribute to the pathology of spinal muscular atrophy models

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Cardiac defects contribute to the pathology of spinal muscular atrophy models

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prefLabel

Cardiac defects contribute to the pathology of spinal muscular atrophy models

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Cardiac defects contribute to the pathology of spinal muscular atrophy models

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Cardiac defects contribute to the pathology of spinal muscular atrophy models

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P1433

Human Molecular Genetics

P1476

Cardiac defects contribute to the pathology of spinal muscular atrophy models

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P2093

Christian L Lorson

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

Hsiao T Yang

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

Javad Habibi

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

Monir Shababi

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Spencer M Vale

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Will A Sewell

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P2860

Development of a single vector system that enhances trans-splicing of SMN2 transcripts

Method for non-invasively recording electrocardiograms in conscious mice

Imbalance between xanthine oxidase and nitric oxide synthase signaling pathways underlies mechanoenergetic uncoupling in the failing heart.

Altered focal adhesion regulation correlates with cardiomyopathy in mice expressing constitutively active rac1

Congenital heart disease is a feature of severe infantile spinal muscular atrophy

Medical considerations of long-term survival of Werdnig-Hoffmann disease

Molecular characterization of angiotensin II--induced hypertrophy of cardiac myocytes and hyperplasia of cardiac fibroblasts. Critical role of the AT1 receptor subtype

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

Translational readthrough by the aminoglycoside geneticin (G418) modulates SMN stability in vitro and improves motor function in SMA mice in vivo

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scholarly article

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449839

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10.1093/HMG/DDQ329

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20

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19

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2010-10-15T00:00:00Z

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45583124

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20696672

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P921

spinal muscular atrophy