Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients

scientific article published on 4 April 2011

Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients is …
instance of (P31):
scholarly articleQ13442814

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P6179Dimensions Publication ID1036381811
P356DOI10.1186/2044-5040-1-15
P932PMC publication ID3156638
P698PubMed publication ID21798095
P5875ResearchGate publication ID51529528

P50authorSilvère M van der MaarelQ59661580
Vered RazQ87734521
John VissingQ90104169
Baziel van EngelenQ110544835
Seyed Yahya AnvarQ114417603
Andrea VenemaQ117249159
Barbara van der SluijsQ130279306
Gert-Jan van OmmenQ29643727
Capucine TrolletQ42323052
Peter A.C. 't HoenQ57190543
P2093author name stringMartine Simonelig
Aymeric Chartier
George Dickson
Marc Snoeck
P2860cites workGenome-wide and functional annotation of human E3 ubiquitin ligases identifies MULAN, a mitochondrial E3 that regulates the organelle's dynamics and signalingQ21144354
Short GCG expansions in the PABP2 gene cause oculopharyngeal muscular dystrophyQ24308826
Regulation and cellular roles of ubiquitin-specific deubiquitinating enzymesQ24645701
DAVID: Database for Annotation, Visualization, and Integrated DiscoveryQ27499374
Systematic and integrative analysis of large gene lists using DAVID bioinformatics resourcesQ27860739
Linear models and empirical bayes methods for assessing differential expression in microarray experimentsQ27860758
Nuclear inclusions in oculopharyngeal dystrophyQ72843808
Oculopharyngeal muscular dystrophy. A familial disease of late life characterized by dysphagia and progressive ptosis of the evelidsQ79698970
Nuclear inclusions in oculopharyngeal muscular dystrophy consist of poly(A) binding protein 2 aggregates which sequester poly(A) RNAQ28139419
Normalization of cDNA microarray dataQ28181692
The oculopharyngeal muscular dystrophy locus maps to the region of the cardiac alpha and beta myosin heavy chain genes on chromosome 14q11.2-q13Q28304113
Repeatability of published microarray gene expression analysesQ28307780
Identification of ubiquitin ligases required for skeletal muscle atrophyQ28582211
Recognition and processing of ubiquitin-protein conjugates by the proteasomeQ29547616
Gene expression profile of aging and its retardation by caloric restrictionQ29614554
The ubiquitin-proteasome proteolytic pathway in heart vs skeletal muscle: effects of acute diabetesQ30306225
Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibresQ34102486
Doxycycline attenuates and delays toxicity of the oculopharyngeal muscular dystrophy mutation in transgenic miceQ34415166
A Drosophila model of oculopharyngeal muscular dystrophy reveals intrinsic toxicity of PABPN1.Q34518248
Signaling in muscle atrophy and hypertrophyQ34593198
Progress in understanding the pathogenesis of oculopharyngeal muscular dystrophy.Q35079093
The ubiquitin proteasome system in neurodegenerative diseases: sometimes the chicken, sometimes the egg.Q35558459
Proteasome and peptidase function in MHC-class-I-mediated antigen presentationQ35634031
The role of ubiquitin-proteasome-dependent proteolysis in the remodelling of skeletal muscle.Q35856003
Ubiquitin-protein ligases in muscle wastingQ36241893
Anni 2.0: a multipurpose text-mining tool for the life sciencesQ36787149
Skeletal muscle proteolysis in aging.Q37341420
Targeting the ubiquitin system in cancer therapyQ37424802
Trehalose reduces aggregate formation and delays pathology in a transgenic mouse model of oculopharyngeal muscular dystrophyQ38318433
A global test for groups of genes: testing association with a clinical outcomeQ38522319
In vivo aggregation properties of the nuclear poly(A)-binding protein PABPN1.Q40438611
PABPN1 overexpression leads to upregulation of genes encoding nuclear proteins that are sequestered in oculopharyngeal muscular dystrophy nuclear inclusions.Q40449021
Involvement of the ubiquitin-proteasome pathway and molecular chaperones in oculopharyngeal muscular dystrophyQ44565973
Interferon gamma enhances proteasome activity in recombinant Hep G2 cells that express cytochrome P4502E1: modulation by ethanolQ44569024
Altered proteasome structure, function, and oxidation in aged muscleQ45242260
Sirtuin inhibition protects from the polyalanine muscular dystrophy protein PABPN1.Q46656918
Cognitive impairment and reduced life span of oculopharyngeal muscular dystrophy homozygotesQ47862955
Changes in lamina structure are followed by spatial reorganization of heterochromatic regions in caspase-8-activated human mesenchymal stem cells.Q50714075
Prevention of oculopharyngeal muscular dystrophy by muscular expression of Llama single-chain intrabodies in vivo.Q51788354
Myopathy phenotype in transgenic mice expressing mutated PABPN1 as a model of oculopharyngeal muscular dystrophy.Q52097209
Progressive skeletal muscle weakness in transgenic mice expressing CTG expansions is associated with the activation of the ubiquitin–proteasome pathwayQ57673583
P4510describes a project that usesImageJQ1659584
limmaQ112236343
P433issue1
P407language of work or nameEnglishQ1860
P921main subjectpathologyQ7208
ubiquitin-proteasome systemQ47175589
P304page(s)15
P577publication date2011-04-04
P1433published inSkeletal MuscleQ27723741
P1476titleDeregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patients
P478volume1

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cites work (P2860)
Q37610585"Get the Balance Right": Pathological Significance of Autophagy Perturbation in Neuromuscular Disorders
Q37298886A decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle aging
Q40629006Cytokine genes as potential biomarkers for muscle weakness in OPMD.
Q64236077Deacetylation Inhibition Reverses PABPN1-Dependent Muscle Wasting
Q42907623Dysfunctional transcripts are formed by alternative polyadenylation in OPMD.
Q91843569Inhibition of myostatin improves muscle atrophy in oculopharyngeal muscular dystrophy (OPMD)
Q34071347Interspecies translation of disease networks increases robustness and predictive accuracy.
Q37625578Maintenance of muscle mass and load-induced growth in Muscle RING Finger 1 null mice with age.
Q35093811Major aging-associated RNA expressions change at two distinct age-positions.
Q35224241Mitochondrial dysfunction reveals the role of mRNA poly(A) tail regulation in oculopharyngeal muscular dystrophy pathogenesis
Q38575523Modeling oculopharyngeal muscular dystrophy in myotube cultures reveals reduced accumulation of soluble mutant PABPN1 protein
Q61448849Normal Ribosomal Biogenesis but Shortened Protein Synthetic Response to Acute Eccentric Resistance Exercise in Old Skeletal Muscle
Q50962265Novel mouse models of oculopharyngeal muscular dystrophy (OPMD) reveal early onset mitochondrial defects and suggest loss of PABPN1 may contribute to pathology.
Q34788237Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy
Q37507541Nuclear poly(A)-binding protein aggregates misplace a pre-mRNA outside of SC35 speckle causing its abnormal splicing
Q34449597Oculopharyngeal muscular dystrophy as a paradigm for muscle aging
Q36010483PABPN1-Dependent mRNA Processing Induces Muscle Wasting.
Q38100286PABPN1: molecular function and muscle disease
Q36305636Poly(A) binding protein nuclear 1 levels affect alternative polyadenylation
Q35789502RNA-binding protein misregulation in microsatellite expansion disorders
Q35790486The Inhibition of Heat Shock Protein 90 Facilitates the Degradation of Poly-Alanine Expanded Poly (A) Binding Protein Nuclear 1 via the Carboxyl Terminus of Heat Shock Protein 70-Interacting Protein

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