A Drosophila model of oculopharyngeal muscular dystrophy reveals intrinsic toxicity of PABPN1.
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Model organisms in the fight against muscular dystrophy: lessons from drosophila and ZebrafishTransgenic Drosophila for Investigating DUX4 and FRG1, Two Genes Associated with Facioscapulohumeral Muscular Dystrophy (FSHD)Structural basis for RNA recognition by a type II poly(A)-binding proteinPolyalanine-independent conformational conversion of nuclear poly(A)-binding protein 1 (PABPN1).Hsp70 chaperones and type I PRMTs are sequestered at intranuclear inclusions caused by polyalanine expansions in PABPN1The evolutionarily conserved RNA binding protein SMOOTH is essential for maintaining normal muscle functionLoss of nuclear poly(A)-binding protein 1 causes defects in myogenesis and mRNA biogenesis.Expression of human amyloid precursor protein in the skeletal muscles of Drosophila results in age- and activity-dependent muscle weakness.An integrative approach to ortholog prediction for disease-focused and other functional studies.Getting folded: chaperone proteins in muscle development, maintenance and disease.Interspecies translation of disease networks increases robustness and predictive accuracy.Over-expression of BCL2 rescues muscle weakness in a mouse model of oculopharyngeal muscular dystrophy.Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophyFlightless flies: Drosophila models of neuromuscular disease.A yeast model for polyalanine-expansion aggregation and toxicityMitochondrial dysfunction reveals the role of mRNA poly(A) tail regulation in oculopharyngeal muscular dystrophy pathogenesisRNA-binding protein misregulation in microsatellite expansion disordersRAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndromeThin, a Trim32 ortholog, is essential for myofibril stability and is required for the integrity of the costamere in Drosophila.A decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle agingAn Antibody to Detect Alanine-Expanded PABPN1: A New Tool to Study Oculopharyngeal Muscular DystrophyPABPN1 gene therapy for oculopharyngeal muscular dystrophyPABPN1: molecular function and muscle disease.Cellular maintenance of nuclear protein homeostasisA folded and functional protein domain in an amyloid-like fibril.Modeling oculopharyngeal muscular dystrophy in myotube cultures reveals reduced accumulation of soluble mutant PABPN1 proteinA conserved role for the zinc finger polyadenosine RNA binding protein, ZC3H14, in control of poly(A) tail length.Protein quality control in the nucleus.Regulated Intron Retention and Nuclear Pre-mRNA Decay Contribute to PABPN1 AutoregulationCytokine genes as potential biomarkers for muscle weakness in OPMD.Silencing of drpr leads to muscle and brain degeneration in adult Drosophila.In vivo interaction proteomics reveal a novel p38 mitogen-activated protein kinase/Rack1 pathway regulating proteostasis in Drosophila muscle.Interactions between homopolymeric amino acids (HPAAs).Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients.Wild-type PABPN1 is anti-apoptotic and reduces toxicity of the oculopharyngeal muscular dystrophy mutation.Novel mouse models of oculopharyngeal muscular dystrophy (OPMD) reveal early onset mitochondrial defects and suggest loss of PABPN1 may contribute to pathology.Intranuclear Aggregates Precede Clinical Onset in Oculopharyngeal Muscular Dystrophy.Bruno-3 regulates sarcomere component expression and contributes to muscle phenotypes of myotonic dystrophy type 1.
P2860
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P2860
A Drosophila model of oculopharyngeal muscular dystrophy reveals intrinsic toxicity of PABPN1.
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2006 nî lūn-bûn
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2006 թուականի Ապրիլին հրատարակուած գիտական յօդուած
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2006 թվականի ապրիլին հրատարակված գիտական հոդված
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2006年の論文
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2006年学术文章
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2006年学术文章
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2006年学术文章
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2006年学术文章
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2006年学术文章
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2006年學術文章
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name
A Drosophila model of oculopha ...... intrinsic toxicity of PABPN1.
@ast
A Drosophila model of oculopha ...... intrinsic toxicity of PABPN1.
@en
A Drosophila model of oculopha ...... intrinsic toxicity of PABPN1.
@nl
type
label
A Drosophila model of oculopha ...... intrinsic toxicity of PABPN1.
@ast
A Drosophila model of oculopha ...... intrinsic toxicity of PABPN1.
@en
A Drosophila model of oculopha ...... intrinsic toxicity of PABPN1.
@nl
prefLabel
A Drosophila model of oculopha ...... intrinsic toxicity of PABPN1.
@ast
A Drosophila model of oculopha ...... intrinsic toxicity of PABPN1.
@en
A Drosophila model of oculopha ...... intrinsic toxicity of PABPN1.
@nl
P2093
P2860
P356
P1433
P1476
A Drosophila model of oculopha ...... intrinsic toxicity of PABPN1.
@en
P2093
Aymeric Chartier
Béatrice Benoit
Martine Simonelig
P2860
P304
P356
10.1038/SJ.EMBOJ.7601117
P407
P577
2006-04-27T00:00:00Z