Spinal muscular atrophy and a model for survival of motor neuron protein function in axonal ribonucleoprotein complexes
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The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophySpinal muscular atrophy: development and implementation of potential treatmentsSplicing therapy for neuromuscular diseaseThe survival of motor neuron (SMN) protein interacts with the mRNA-binding protein HuD and regulates localization of poly(A) mRNA in primary motor neuron axons.SMN in spinal muscular atrophy and snRNP biogenesisHigh-efficiency transfection of cultured primary motor neurons to study protein localization, trafficking, and function.Molecular determinants of survival motor neuron (SMN) protein cleavage by the calcium-activated protease, calpainInvertebrate models of spinal muscular atrophy: insights into mechanisms and potential therapeutics.Conserved requirement for DEAD-box RNA helicase Gemin3 in Drosophila oogenesisTemporal requirement for high SMN expression in SMA miceSMN-inducing compounds for the treatment of spinal muscular atrophy.GEMINs: potential therapeutic targets for spinal muscular atrophy?Presynaptic localization of Smn and hnRNP R in axon terminals of embryonic and postnatal mouse motoneuronsInsights into the evolutionary features of human neurodegenerative diseases.Identification of the phosphorylation sites in the survival motor neuron protein by protein kinase A.Splicing inhibition of U2AF65 leads to alternative exon skipping.Survival motor neuron affects plastin 3 protein levels leading to motor defectsSpinal muscular atrophy: the role of SMN in axonal mRNA regulation.Ubiquitin-specific protease 9x deubiquitinates and stabilizes the spinal muscular atrophy protein-survival motor neuronImplication of the SMN complex in the biogenesis and steady state level of the signal recognition particle.Deficiency of the Survival of Motor Neuron Protein Impairs mRNA Localization and Local Translation in the Growth Cone of Motor Neurons.Large-Scale Production of Adeno-Associated Viral Vector Serotype-9 Carrying the Human Survival Motor Neuron Gene.Dysregulation of mRNA Localization and Translation in Genetic DiseaseStructural basis for specific recognition of pre-snRNA by Gemin5.Dynamics of survival of motor neuron (SMN) protein interaction with the mRNA-binding protein IMP1 facilitates its trafficking into motor neuron axons.Electrophysiological Biomarkers in Spinal Muscular Atrophy: Preclinical Proof of Concept.The role of RNA processing in the pathogenesis of motor neuron degeneration.Biomarkers in rare disorders: the experience with spinal muscular atrophy.mRNA localization: an orchestration of assembly, traffic and synthesis.Linking amyotrophic lateral sclerosis and spinal muscular atrophy through RNA-transcriptome homeostasis: a genomics perspective.Spatially and temporally regulating translation via mRNA-binding proteins in cellular and neuronal function.Smn-Deficiency Increases the Intrinsic Excitability of Motoneurons.A new cis-acting motif is required for the axonal SMN-dependent Anxa2 mRNA localization.mRNP assembly, axonal transport, and local translation in neurodegenerative diseases.Axon outgrowth and neuronal differentiation defects after a-SMN and FL-SMN silencing in primary hippocampal cultures.
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Spinal muscular atrophy and a model for survival of motor neuron protein function in axonal ribonucleoprotein complexes
description
article científic
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article scientifique
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articolo scientifico
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artigo científico
@pt
bilimsel makale
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scientific article published on January 2009
@en
vedecký článok
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vetenskaplig artikel
@sv
videnskabelig artikel
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vědecký článek
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name
Spinal muscular atrophy and a ...... al ribonucleoprotein complexes
@en
Spinal muscular atrophy and a ...... l ribonucleoprotein complexes.
@nl
type
label
Spinal muscular atrophy and a ...... al ribonucleoprotein complexes
@en
Spinal muscular atrophy and a ...... l ribonucleoprotein complexes.
@nl
prefLabel
Spinal muscular atrophy and a ...... al ribonucleoprotein complexes
@en
Spinal muscular atrophy and a ...... l ribonucleoprotein complexes.
@nl
P2860
P356
P1476
Spinal muscular atrophy and a ...... al ribonucleoprotein complexes
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P2093
Gary J Bassell
P2860
P304
P356
10.1007/400_2009_4
P577
2009-01-01T00:00:00Z