Defective Neuromuscular Junction Organization and Postnatal Myogenesis in Mice With Severe Spinal Muscular Atrophy
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Is spinal muscular atrophy a disease of the motor neurons only: pathogenesis and therapeutic implications?Skeletal muscle DNA damage precedes spinal motor neuron DNA damage in a mouse model of Spinal Muscular Atrophy (SMA)Immune dysregulation may contribute to disease pathogenesis in spinal muscular atrophy miceThe lack of CuZnSOD leads to impaired neurotransmitter release, neuromuscular junction destabilization and reduced muscle strength in mice.Splicing changes in SMA mouse motoneurons and SMN-depleted neuroblastoma cells: evidence for involvement of splicing regulatory proteins.Neurofilament Phosphorylation during Development and Disease: Which Came First, the Phosphorylation or the Accumulation?Neurogenic and myogenic contributions to hereditary motor neuron disease.Pre- and postsynaptic changes in the neuromuscular junction in dystrophic mice.Transcriptional profiling of differentially vulnerable motor neurons at pre-symptomatic stage in the Smn (2b/-) mouse model of spinal muscular atrophy.Copy number variations in 6q14.1 and 5q13.2 are associated with alcohol dependenceMechanistic principles of antisense targets for the treatment of spinal muscular atrophyChronic 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.Notch signaling pathway is activated in motoneurons of spinal muscular atrophy.Survival Motor Neuron (SMN) protein is required for normal mouse liver development.Spinal muscular atrophy astrocytes exhibit abnormal calcium regulation and reduced growth factor productionSpinal muscular atrophy: new findings for an old pathology.Genome-wide analysis shows association of epigenetic changes in regulators of Rab and Rho GTPases with spinal muscular atrophy severity.New insights into SMA pathogenesis: immune dysfunction and neuroinflammation.SMN deficiency does not induce oxidative stress in SMA iPSC-derived astrocytes or motor neurons.Neuroprotective Effect of Non-viral Gene Therapy Treatment Based on Tetanus Toxin C-fragment in a Severe Mouse Model of Spinal Muscular AtrophyAstrocytes influence the severity of spinal muscular atrophy.The craniosacral progression of muscle development influences the emergence of neuromuscular junction alterations in a severe murine model for spinal muscular atrophy.Myogenic program dysregulation is contributory to disease pathogenesis in spinal muscular atrophy.Imaging Analysis of the Neuromuscular Junction in Dystrophic Muscle.Synaptic defects in type I spinal muscular atrophy in human development.Identification of RNA bound to the TDP-43 ribonucleoprotein complex in the adult mouse brain.Survival of motor neurone protein is required for normal postnatal development of the spleen.THEME 11 THERAPEUTIC STRATEGIES
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P2860
Defective Neuromuscular Junction Organization and Postnatal Myogenesis in Mice With Severe Spinal Muscular Atrophy
description
im Juni 2011 veröffentlichter wissenschaftlicher Artikel
@de
scientific article published on 01 June 2011
@en
wetenschappelijk artikel
@nl
наукова стаття, опублікована в червні 2011
@uk
name
Defective Neuromuscular Juncti ...... Severe Spinal Muscular Atrophy
@en
Defective Neuromuscular Juncti ...... Severe Spinal Muscular Atrophy
@nl
type
label
Defective Neuromuscular Juncti ...... Severe Spinal Muscular Atrophy
@en
Defective Neuromuscular Juncti ...... Severe Spinal Muscular Atrophy
@nl
prefLabel
Defective Neuromuscular Juncti ...... Severe Spinal Muscular Atrophy
@en
Defective Neuromuscular Juncti ...... Severe Spinal Muscular Atrophy
@nl
P2093
P2860
P1476
Defective neuromuscular juncti ...... severe spinal muscular atrophy
@en
P2093
Anna Casanovas
Elisabet Dachs
Lídia Piedrafita
Marta Hereu
P2860
P304
P356
10.1097/NEN.0B013E31821CBD8B
P577
2011-06-01T00:00:00Z