Cardiac defects contribute to the pathology of spinal muscular atrophy models
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Spinal muscular atrophyThe contribution of mouse models to understanding the pathogenesis of spinal muscular atrophyIs 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 biogenesisPostsymptomatic 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 SplicingEmerging therapies and challenges in spinal muscular atrophyImmune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy miceThe 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 lateA short antisense oligonucleotide ameliorates symptoms of severe mouse models of spinal muscular atrophyROCK inhibition as a therapy for spinal muscular atrophy: understanding the repercussions on multiple cellular targetsTemporal requirement for high SMN expression in SMA miceSystemic gene delivery in large species for targeting spinal cord, brain, and peripheral tissues for pediatric disordersNew therapeutic approaches to spinal muscular atrophy.A single administration of morpholino antisense oligomer rescues spinal muscular atrophy in mouseSMN-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 miceSpinal muscular atrophy: from tissue specificity to therapeutic strategiesMotor neuron cell-nonautonomous rescue of spinal muscular atrophy phenotypes in mild and severe transgenic mouse modelsGlucose metabolism and pancreatic defects in spinal muscular atrophyMuscles 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 neuronsPeripheral 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 deliveryPartial 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
P2860
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P2860
Cardiac defects contribute to the pathology of spinal muscular atrophy models
description
2010 nî lūn-bûn
@nan
2010 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Cardiac defects contribute to the pathology of spinal muscular atrophy models
@ast
Cardiac defects contribute to the pathology of spinal muscular atrophy models
@en
Cardiac defects contribute to the pathology of spinal muscular atrophy models
@nl
type
label
Cardiac defects contribute to the pathology of spinal muscular atrophy models
@ast
Cardiac defects contribute to the pathology of spinal muscular atrophy models
@en
Cardiac defects contribute to the pathology of spinal muscular atrophy models
@nl
prefLabel
Cardiac defects contribute to the pathology of spinal muscular atrophy models
@ast
Cardiac defects contribute to the pathology of spinal muscular atrophy models
@en
Cardiac defects contribute to the pathology of spinal muscular atrophy models
@nl
P2093
P2860
P3181
P356
P1476
Cardiac defects contribute to the pathology of spinal muscular atrophy models
@en
P2093
Christian L Lorson
Hsiao T Yang
Javad Habibi
Monir Shababi
Spencer M Vale
Will A Sewell
P2860
P304
P3181
P356
10.1093/HMG/DDQ329
P407
P577
2010-10-15T00:00:00Z