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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The 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?Spinal muscular atrophy: development and implementation of potential treatmentsSkeletal muscle DNA damage precedes spinal motor neuron DNA damage in a mouse model of Spinal Muscular Atrophy (SMA)Synaptic defects in the spinal and neuromuscular circuitry in a mouse model of spinal muscular atrophyA large animal model of spinal muscular atrophy and correction of phenotype.Utility of survival motor neuron ELISA for spinal muscular atrophy clinical and preclinical analysesPostsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy.SMN is required for sensory-motor circuit function in Drosophila.Temporal requirement for SMN in motoneuron development.Pathological impact of SMN2 mis-splicing in adult SMA miceRequirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation.Sensory neurons do not induce motor neuron loss in a human stem cell model of spinal muscular atrophySMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminalsTranscriptome profiling of spinal muscular atrophy motor neurons derived from mouse embryonic stem cellsTemporal requirement for high SMN expression in SMA miceAntisense 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.Increased IGF-1 in muscle modulates the phenotype of severe SMA miceSpinal muscular atrophy: diagnosis and management in a new therapeutic eraImprovement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neuronsSpinal muscular atrophy: from tissue specificity to therapeutic strategiesMotor neuron cell-nonautonomous rescue of spinal muscular atrophy phenotypes in mild and severe transgenic mouse modelsMuscles 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 neuronsSpinal muscular atrophy: journeying from bench to bedsideThe Genetics of Spinal Muscular Atrophy: Progress and Challenges.Severe neuromuscular denervation of clinically relevant muscles in a mouse model of spinal muscular atrophy.Disease mechanisms and therapeutic approaches in spinal muscular atrophy.Survival motor neuron affects plastin 3 protein levels leading to motor defectsNeurogenic and myogenic contributions to hereditary motor neuron disease.SMN expression is required in motor neurons to rescue electrophysiological deficits in the SMNΔ7 mouse model of SMA.TSUNAMI: an antisense method to phenocopy splicing-associated diseases in animals.Characterization of behavioral and neuromuscular junction phenotypes in a novel allelic series of SMA mouse modelsSurvival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophyα-COP binding to the survival motor neuron protein SMN is required for neuronal process outgrowthBehavioral and electrophysiological outcomes of tissue-specific Smn knockdown in Drosophila melanogaster.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.Preservation of VGLUT1 synapses on ventral calbindin-immunoreactive interneurons and normal locomotor function in a mouse model of spinal muscular atrophyMotor neuron rescue in spinal muscular atrophy mice demonstrates that sensory-motor defects are a consequence, not a cause, of motor neuron dysfunctionTranscriptional enhancement of Smn levels in motoneurons is crucial for proper axon morphology in zebrafish.
P2860
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P2860
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
description
2010 nî lūn-bûn
@nan
2010 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի սեպտեմբերին 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
Reduced survival of motor neur ...... human centromeric (SMN2) gene
@ast
Reduced survival of motor neur ...... human centromeric (SMN2) gene
@en
Reduced survival of motor neuron
@nl
type
label
Reduced survival of motor neur ...... human centromeric (SMN2) gene
@ast
Reduced survival of motor neur ...... human centromeric (SMN2) gene
@en
Reduced survival of motor neuron
@nl
prefLabel
Reduced survival of motor neur ...... human centromeric (SMN2) gene
@ast
Reduced survival of motor neur ...... human centromeric (SMN2) gene
@en
Reduced survival of motor neuron
@nl
P2093
P2860
P1476
Reduced survival of motor neur ...... human centromeric (SMN2) gene
@en
P2093
Gyu-Hwan Park
Lynn T Landmesser
Tomoyuki Awano
Umrao R Monani
Yuka Maeno-Hikichi
P2860
P304
12005-12019
P356
10.1523/JNEUROSCI.2208-10.2010
P407
P577
2010-09-01T00:00:00Z