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Survival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophy

Survival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophy

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Neuromuscular Junctions as Key Contributors and Therapeutic Targets in Spinal Muscular Atrophy Is spinal muscular atrophy a disease of the motor neurons only: pathogenesis and therapeutic implications?Spinal muscular atrophy: development and implementation of potential treatments Small molecule suppressors of Drosophila kinesin deficiency rescue motor axon development in a zebrafish model of spinal muscular atrophy SMN is required for sensory-motor circuit function in Drosophila.Protective effects of butyrate-based compounds on a mouse model for spinal muscular atrophy.Efficient SMN Rescue following Subcutaneous Tricyclo-DNA Antisense Oligonucleotide Treatment Muscle matters in Kennedy's disease.Muscle expression of mutant androgen receptor accounts for systemic and motor neuron disease phenotypes in spinal and bulbar muscular atrophy Sensory neurons do not induce motor neuron loss in a human stem cell model of spinal muscular atrophy Shared mechanisms between Drosophila peripheral nervous system development and human neurodegenerative diseases Survival motor neuron protein deficiency impairs myotube formation by altering myogenic gene expression and focal adhesion dynamics.Transcriptome profiling of spinal muscular atrophy motor neurons derived from mouse embryonic stem cells Antisense oligonucleotides for the treatment of spinal muscular atrophy.Presynaptic localization of Smn and hnRNP R in axon terminals of embryonic and postnatal mouse motoneurons Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy.Spinal muscular atrophy: diagnosis and management in a new therapeutic era Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons 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 Spinal muscular atrophy: journeying from bench to bedside The Genetics of Spinal Muscular Atrophy: Progress and Challenges.Plastin 3 Expression Does Not Modify Spinal Muscular Atrophy Severity in the ∆7 SMA Mouse Disease mechanisms and therapeutic approaches 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.A Comparative Study of SMN Protein and mRNA in Blood and Fibroblasts in Patients with Spinal Muscular Atrophy and Healthy Controls Decay in survival motor neuron and plastin 3 levels during differentiation of iPSC-derived human motor neurons.Genetic inhibition of JNK3 ameliorates spinal muscular atrophy.α-COP binding to the survival motor neuron protein SMN is required for neuronal process outgrowth Behavioral 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 atrophy Deficiency of the Survival of Motor Neuron Protein Impairs mRNA Localization and Local Translation in the Growth Cone of Motor Neurons.Transcriptional enhancement of Smn levels in motoneurons is crucial for proper axon morphology in zebrafish.The DcpS inhibitor RG3039 improves motor function in SMA mice.More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases.A Novel Striated Muscle-Specific Myosin-Blocking Drug for the Study of Neuromuscular Physiology.SMN-targeted therapeutics for spinal muscular atrophy: are we SMArt enough yet?

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Survival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophy

http://www.wikidata.org/entity/Q36292754

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2012年學術文章

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2012年學術文章

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2012年學術文章

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name

Survival motor neuron protein ...... ity in spinal muscular atrophy

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Survival motor neuron protein ...... ity in spinal muscular atrophy

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type

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Survival motor neuron protein ...... ity in spinal muscular atrophy

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Survival motor neuron protein ...... ity in spinal muscular atrophy

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Survival motor neuron protein ...... ity in spinal muscular atrophy

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Survival motor neuron protein ...... ity in spinal muscular atrophy

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JNEUROSCI.0204-12.2012

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Journal of Neuroscience

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Survival motor neuron protein ...... ity in spinal muscular atrophy

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Cathleen M Lutz

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Charlotte J Sumner

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Crystal Davis

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David J Goldhamer

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James P Van Meerbeke

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Joe Wooley

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Lingling Kong

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Mark M Rich

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Melissa A Osborne

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Melissa E Crowder

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P2860

Modeling spinal muscular atrophy in Drosophila

The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy

Delivery of recombinant follistatin lessens disease severity in a mouse model of spinal muscular atrophy.

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

Generalized lacZ expression with the ROSA26 Cre reporter strain

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

Arrhythmia and cardiac defects are a feature of spinal muscular atrophy model mice.

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

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

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8703-8715

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scholarly article

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10.1523/JNEUROSCI.0204-12.2012

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25

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32

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2012-06-01T00:00:00Z

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227858136

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22723710

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spinal muscular atrophy

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3462658

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