Nuclear inclusions in oculopharyngeal muscular dystrophy consist of poly(A) binding protein 2 aggregates which sequester poly(A) RNA
about
Promiscuous modification of the nuclear poly(A)-binding protein by multiple protein-arginine methyltransferases does not affect the aggregation behavior3' end mRNA processing: molecular mechanisms and implications for health and diseasePoly(A)-binding proteins: multifunctional scaffolds for the post-transcriptional control of gene expressionProcessing and transcriptome expansion at the mRNA 3' end in health and disease: finding the right endRNA-binding proteins and gene regulation in myogenesisHnRNP A1 and A/B interaction with PABPN1 in oculopharyngeal muscular dystrophyMammalian, yeast, bacterial, and chemical chaperones reduce aggregate formation and death in a cell model of oculopharyngeal muscular dystrophyFacioscapulohumeral muscular dystrophy in mice overexpressing FRG1Oculopharyngeal muscular dystrophy: a polyalanine myopathyPolyadenylation-dependent control of long noncoding RNA expression by the poly(A)-binding protein nuclear 1Polyalanine-independent conformational conversion of nuclear poly(A)-binding protein 1 (PABPN1).Frequent missense and insertion/deletion polymorphisms in the ovine Shadoo gene parallel species-specific variation in PrP.Loss of nuclear poly(A)-binding protein 1 causes defects in myogenesis and mRNA biogenesis.Influence of the stability of a fused protein and its distance to the amyloidogenic segment on fibril formation.Doxycycline attenuates and delays toxicity of the oculopharyngeal muscular dystrophy mutation in transgenic mice.Comparative analysis of the Shadoo gene between cattle and buffalo reveals significant differences.Oculopharyngeal muscular dystrophy as a paradigm for muscle aging.A Drosophila model of oculopharyngeal muscular dystrophy reveals intrinsic toxicity of PABPN1.Depletion of nuclear poly(A) binding protein PABPN1 produces a compensatory response by cytoplasmic PABP4 and PABP5 in cultured human cells.Abnormalities of the nucleus and nuclear inclusions in neurodegenerative disease: a work in progress.Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophyMechanistic insight into the pathology of polyalanine expansion disorders revealed by a mouse model for X linked hypopituitarismPhylogenetic and molecular characterization of the splicing factor RBM4.Alanine repeats influence protein localization in splicing speckles and paraspecklesNuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy.A yeast model for polyalanine-expansion aggregation and toxicityPoly(A) RNA-binding proteins and polyadenosine RNA: new members and novel functions.Mitochondrial dysfunction reveals the role of mRNA poly(A) tail regulation in oculopharyngeal muscular dystrophy pathogenesisProgressive myopathy in an inducible mouse model of oculopharyngeal muscular dystrophy.Reducing bodies and myofibrillar myopathy features in FHL1 muscular dystrophy.PABPN1-Dependent mRNA Processing Induces Muscle Wasting.Diagnostic immunohistochemistry in neuromuscular disorders.Implications of polyadenylation in health and disease.Poly(A) binding protein nuclear 1 levels affect alternative polyadenylation.A polyalanine tract expansion in Arx forms intranuclear inclusions and results in increased cell death.Alternative splicing and muscular dystrophy.Dynamic mutations as digital genetic modulators of brain development, function and dysfunction.RNA-binding proteins in neurodegenerative disease: TDP-43 and beyond.Oculopharyngeal Weakness, Hypophrenia, Deafness, and Impaired Vision: A Novel Autosomal Dominant Myopathy with Rimmed VacuolesAmyloidogenesis of natively unfolded proteins.
P2860
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P2860
Nuclear inclusions in oculopharyngeal muscular dystrophy consist of poly(A) binding protein 2 aggregates which sequester poly(A) RNA
description
2000 nî lūn-bûn
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2000 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի սեպտեմբերին հրատարակված գիտական հոդված
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2000年の論文
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2000年論文
@yue
2000年論文
@zh-hant
2000年論文
@zh-hk
2000年論文
@zh-mo
2000年論文
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2000年论文
@wuu
name
Nuclear inclusions in oculopha ...... es which sequester poly(A) RNA
@ast
Nuclear inclusions in oculopha ...... es which sequester poly(A) RNA
@en
Nuclear inclusions in oculopha ...... es which sequester poly(A) RNA
@nl
type
label
Nuclear inclusions in oculopha ...... es which sequester poly(A) RNA
@ast
Nuclear inclusions in oculopha ...... es which sequester poly(A) RNA
@en
Nuclear inclusions in oculopha ...... es which sequester poly(A) RNA
@nl
prefLabel
Nuclear inclusions in oculopha ...... es which sequester poly(A) RNA
@ast
Nuclear inclusions in oculopha ...... es which sequester poly(A) RNA
@en
Nuclear inclusions in oculopha ...... es which sequester poly(A) RNA
@nl
P2093
P3181
P1476
Nuclear inclusions in oculopha ...... es which sequester poly(A) RNA
@en
P2093
G A Rouleau
M Carmo-Fonseca
P304
P3181
P356
10.1093/OXFORDJOURNALS.HMG.A018924
P407
P577
2000-09-22T00:00:00Z