A Drosophila model of spinal muscular atrophy uncouples snRNP biogenesis functions of survival motor neuron from locomotion and viability defects.
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The survival motor neuron protein forms soluble glycine zipper oligomersSplicing therapy for neuromuscular diseaseChondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophyThe Survival of Motor Neuron Protein Acts as a Molecular Chaperone for mRNP AssemblyQuality control of assembly-defective U1 snRNAs by decapping and 5'-to-3' exonucleolytic digestion.GEMINs: potential therapeutic targets for spinal muscular atrophy?Subcellular transcriptome alterations in a cell culture model of spinal muscular atrophy point to widespread defects in axonal growth and presynaptic differentiation.The Gemin associates of survival motor neuron are required for motor function in Drosophila.Spinal muscular atrophy: journeying from bench to bedsideThe survival motor neuron gene smn-1 interacts with the U2AF large subunit gene uaf-1 to regulate Caenorhabditis elegans lifespan and motor functions.Genetic inhibition of JNK3 ameliorates spinal muscular atrophy.Behavioral and electrophysiological outcomes of tissue-specific Smn knockdown in Drosophila melanogaster.Ubiquitin-specific protease 9x deubiquitinates and stabilizes the spinal muscular atrophy protein-survival motor neuronIdentification and characterization of Drosophila Snurportin reveals a role for the import receptor Moleskin/importin-7 in snRNP biogenesisThe Power of Human Protective Modifiers: PLS3 and CORO1C Unravel Impaired Endocytosis in Spinal Muscular Atrophy and Rescue SMA PhenotypeDevelopmental arrest of Drosophila survival motor neuron (Smn) mutants accounts for differences in expression of minor intron-containing genes.Dysregulation of ubiquitin homeostasis and β-catenin signaling promote spinal muscular atrophy.SMN deficiency in severe models of spinal muscular atrophy causes widespread intron retention and DNA damageA day in the life of the spliceosomeGem depletion: amyotrophic lateral sclerosis and spinal muscular atrophy crossover.Neurodegeneration in spinal muscular atrophy: from disease phenotype and animal models to therapeutic strategies and beyond.SMN control of RNP assembly: from post-transcriptional gene regulation to motor neuron disease.mRNA metabolism and neuronal disease.Towards an understanding of regulating Cajal body activity by protein modification.Linking amyotrophic lateral sclerosis and spinal muscular atrophy through RNA-transcriptome homeostasis: a genomics perspective.Diverse role of survival motor neuron protein.Developmental regulation of SMN expression: pathophysiological implications and perspectives for therapy development in spinal muscular atrophy.Identification of processing elements and interactors implicate SMN, coilin and the pseudogene-encoded coilp1 in telomerase and box C/D scaRNP biogenesis.Therapeutic strategies for spinal muscular atrophy: SMN and beyond.SMA-causing missense mutations in survival motor neuron (Smn) display a wide range of phenotypes when modeled in Drosophila.An SMN-dependent U12 splicing event essential for motor circuit functionDevelopmental analysis of spliceosomal snRNA isoform expression.Transcriptomic comparison of Drosophila snRNP biogenesis mutants reveals mutant-specific changes in pre-mRNA processing: implications for spinal muscular atrophy.Impaired spliceosomal UsnRNP assembly leads to Sm mRNA down-regulation and Sm protein degradation.Self-oligomerization regulates stability of survival motor neuron protein isoforms by sequestering an SCFSlmb degron.Novel interactors of the Drosophila Survival Motor Neuron (SMN) Complex suggest its full conservation.SMN regulation in SMA and in response to stress: new paradigms and therapeutic possibilities.mRNP assembly, axonal transport, and local translation in neurodegenerative diseases.as a Model for Assessing the Function of RNA-Binding Proteins during Neurogenesis and Neurological Disease
P2860
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P2860
A Drosophila model of spinal muscular atrophy uncouples snRNP biogenesis functions of survival motor neuron from locomotion and viability defects.
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 Drosophila model of spinal m ...... omotion and viability defects.
@ast
A Drosophila model of spinal m ...... omotion and viability defects.
@en
type
label
A Drosophila model of spinal m ...... omotion and viability defects.
@ast
A Drosophila model of spinal m ...... omotion and viability defects.
@en
prefLabel
A Drosophila model of spinal m ...... omotion and viability defects.
@ast
A Drosophila model of spinal m ...... omotion and viability defects.
@en
P2860
P1433
P1476
A Drosophila model of spinal m ...... comotion and viability defects
@en
P2093
Kavita Praveen
P2860
P304
P356
10.1016/J.CELREP.2012.05.014
P577
2012-06-21T00:00:00Z