Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres.
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Loss of a single allele for Ku80 leads to progenitor dysfunction and accelerated aging in skeletal muscleMicrogenomic analysis in skeletal muscle: expression signatures of individual fast and slow myofibersThe ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solutionInterspecies translation of disease networks increases robustness and predictive accuracy.Impaired adaptive response to mechanical overloading in dystrophic skeletal muscleRapid determination of myosin heavy chain expression in rat, mouse, and human skeletal muscle using multicolor immunofluorescence analysis.Oculopharyngeal muscular dystrophy as a paradigm for muscle aging.Over-expression of BCL2 rescues muscle weakness in a mouse model of oculopharyngeal muscular dystrophy.Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy.Mitochondrial dysfunction reveals the role of mRNA poly(A) tail regulation in oculopharyngeal muscular dystrophy pathogenesisAbnormal Skeletal Muscle Regeneration plus Mild Alterations in Mature Fiber Type Specification in Fktn-Deficient Dystroglycanopathy Muscular Dystrophy Mice.PABPN1-Dependent mRNA Processing Induces Muscle Wasting.Differential myofiber-type transduction preference of adeno-associated virus serotypes 6 and 9.Poly(A) binding protein nuclear 1 levels affect alternative polyadenylation.Robust Long-term Transduction of Common Marmoset Neuromuscular Tissue With rAAV1 and rAAV9.A decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle agingNuclear poly(A)-binding protein aggregates misplace a pre-mRNA outside of SC35 speckle causing its abnormal splicing.An 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.Modeling oculopharyngeal muscular dystrophy in myotube cultures reveals reduced accumulation of soluble mutant PABPN1 proteinImmortalized pathological human myoblasts: towards a universal tool for the study of neuromuscular disorders.Cytokine genes as potential biomarkers for muscle weakness in OPMD.An alanine expanded PABPN1 causes increased utilization of intronic polyadenylation sites.Silencing Nfix rescues muscular dystrophy by delaying muscle regeneration.Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients.Dysfunctional transcripts are formed by alternative polyadenylation in OPMD.Proteasomal activity-based probes mark protein homeostasis in muscles.Novel mouse models of oculopharyngeal muscular dystrophy (OPMD) reveal early onset mitochondrial defects and suggest loss of PABPN1 may contribute to pathology.Valproic acid is protective in cellular and worm models of oculopharyngeal muscular dystrophyAtrophy, Fibrosis, and Increased PAX7-Positive Cells in Pharyngeal Muscles of Oculopharyngeal Muscular Dystrophy PatientsEffect of locomotor training on muscle performance in the context of nerve-muscle communication dysfunctionA centronuclear myopathy-dynamin 2 mutation impairs skeletal muscle structure and function in mice
P2860
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P2860
Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibres.
description
2010 nî lūn-bûn
@nan
2010 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի մարտին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Molecular and phenotypic chara ...... ted to fast glycolytic fibres.
@ast
Molecular and phenotypic chara ...... ted to fast glycolytic fibres.
@en
Molecular and phenotypic chara ...... ted to fast glycolytic fibres.
@nl
type
label
Molecular and phenotypic chara ...... ted to fast glycolytic fibres.
@ast
Molecular and phenotypic chara ...... ted to fast glycolytic fibres.
@en
Molecular and phenotypic chara ...... ted to fast glycolytic fibres.
@nl
prefLabel
Molecular and phenotypic chara ...... ted to fast glycolytic fibres.
@ast
Molecular and phenotypic chara ...... ted to fast glycolytic fibres.
@en
Molecular and phenotypic chara ...... ted to fast glycolytic fibres.
@nl
P2093
P50
P356
P1476
Molecular and phenotypic chara ...... ted to fast glycolytic fibres.
@en
P2093
Alban Vignaud
Andrea Venema
Arnaud Ferry
Christophe Hourde
David C Rubinsztein
George Dickson
Iain P Hargreaves
Janet E Davies
Keith Foster
Martin A Baraibar
P304
P356
10.1093/HMG/DDQ098
P407
P577
2010-03-05T00:00:00Z