A cell-autonomous defect in skeletal muscle satellite cells expressing low levels of survival of motor neuron protein.
about
Skeletal muscle DNA damage precedes spinal motor neuron DNA damage in a mouse model of Spinal Muscular Atrophy (SMA)Requirement of enhanced Survival Motoneuron protein imposed during neuromuscular junction maturation.Immune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy miceSurvival motor neuron protein deficiency impairs myotube formation by altering myogenic gene expression and focal adhesion dynamics.Systemic peptide-mediated oligonucleotide therapy improves long-term survival in spinal muscular atrophy.Spinal muscular atrophy: from tissue specificity to therapeutic strategiesMotor neuron cell-nonautonomous rescue of spinal muscular atrophy phenotypes in mild and severe transgenic mouse modelsImproving single injection CSF delivery of AAV9-mediated gene therapy for SMA: a dose-response study in mice and nonhuman primates.Hyperhomocysteinemia inhibits satellite cell regenerative capacity through p38 alpha/beta MAPK signaling.Inducible depletion of adult skeletal muscle stem cells impairs the regeneration of neuromuscular junctions.Low levels of Survival Motor Neuron protein are sufficient for normal muscle function in the SMNΔ7 mouse model of SMA.Activin Receptor Type IIB Inhibition Improves Muscle Phenotype and Function in a Mouse Model of Spinal Muscular AtrophyRole of stem/progenitor cells in reparative disorders.Impaired Muscle Mitochondrial Biogenesis and Myogenesis in Spinal Muscular Atrophy.Regulation of constitutive and alternative splicing by PRMT5 reveals a role for Mdm4 pre-mRNA in sensing defects in the spliceosomal machineryEfficacy and biodistribution analysis of intracerebroventricular administration of an optimized scAAV9-SMN1 vector in a mouse model of spinal muscular atrophy.More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases.Spinal muscular atrophy: a motor neuron disorder or a multi-organ disease?Supraphysiological expression of survival motor neuron protein from an adenovirus vector does not adversely affect cell function.DNA Damage Response and DNA Repair in Skeletal Myocytes From a Mouse Model of Spinal Muscular Atrophy.SMA-causing missense mutations in survival motor neuron (Smn) display a wide range of phenotypes when modeled in Drosophila.Hedgehog-driven myogenic tumors recapitulate skeletal muscle cellular heterogeneity.
P2860
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P2860
A cell-autonomous defect in skeletal muscle satellite cells expressing low levels of survival of motor neuron protein.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
A cell-autonomous defect in sk ...... vival of motor neuron protein.
@en
A cell-autonomous defect in sk ...... vival of motor neuron protein.
@nl
type
label
A cell-autonomous defect in sk ...... vival of motor neuron protein.
@en
A cell-autonomous defect in sk ...... vival of motor neuron protein.
@nl
prefLabel
A cell-autonomous defect in sk ...... vival of motor neuron protein.
@en
A cell-autonomous defect in sk ...... vival of motor neuron protein.
@nl
P2093
P2860
P1476
A cell-autonomous defect in sk ...... rvival of motor neuron protein
@en
P2093
Amanda K Wagner
Amy J Wagers
Massimiliano Cerletti
Monica Hayhurst
P2860
P304
P356
10.1016/J.YDBIO.2012.05.037
P407
P50
P577
2012-06-15T00:00:00Z