A decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle aging

scientific article published on June 2013

A decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle aging is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.18632/AGING.100567
P8608Fatcat IDrelease_plwyrcovrjbotgvlm6vuhlv67i
P932PMC publication ID3824410
P698PubMed publication ID23793615

P50authorBaziel van EngelenQ110544835
Seyed Yahya AnvarQ114417603
Andrea VenemaQ117249159
Barbara van der SluijsQ130279306
Nisha VerwayQ130279323
Jelle J GoemanQ48799316
Yotam RazQ56252230
Peter A.C. 't HoenQ57190543
Silvère M van der MaarelQ59661580
Vered RazQ87734521
John VissingQ90104169
P2860cites workShort GCG expansions in the PABP2 gene cause oculopharyngeal muscular dystrophyQ24308826
The STRING database in 2011: functional interaction networks of proteins, globally integrated and scoredQ24607390
Linear models and empirical bayes methods for assessing differential expression in microarray experimentsQ27860758
Nuclear inclusions in oculopharyngeal muscular dystrophy consist of poly(A) binding protein 2 aggregates which sequester poly(A) RNAQ28139419
Oculopharyngeal muscular dystrophy: a polyalanine myopathyQ28303935
Oculopharyngeal muscular dystrophy-like nuclear inclusions are present in normal magnocellular neurosecretory neurons of the hypothalamusQ28570226
Impairment of the ubiquitin-proteasome system by protein aggregationQ29614556
Literature-aided meta-analysis of microarray data: a compendium study on muscle development and diseaseQ31160383
Loss of nuclear poly(A)-binding protein 1 causes defects in myogenesis and mRNA biogenesis.Q33697689
Molecular and phenotypic characterization of a mouse model of oculopharyngeal muscular dystrophy reveals severe muscular atrophy restricted to fast glycolytic fibresQ34102486
Fat tissue, aging, and cellular senescenceQ34130783
Oxidative damage and mitochondrial decay in agingQ34326443
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
Nuclear and chromatin reorganization during cell senescence and aging - a mini-reviewQ34540621
Polyglutamine diseases: emerging concepts in pathogenesis and therapyQ34696955
Pre-mRNA splicing modulations in senescence.Q35185569
Analysis of alternative splicing associated with aging and neurodegeneration in the human brain.Q35451664
Progressive myopathy in an inducible mouse model of oculopharyngeal muscular dystrophyQ35577123
Nile red: a selective fluorescent stain for intracellular lipid dropletsQ36211849
Opinion: What is the role of protein aggregation in neurodegeneration?Q36259086
Poly(A) binding protein nuclear 1 levels affect alternative polyadenylationQ36305636
Anni 2.0: a multipurpose text-mining tool for the life sciencesQ36787149
The Human Ageing Genomic Resources: online databases and tools for biogerontologists.Q37318261
Intracellular heterogeneity in mitochondrial membrane potentials revealed by a J-aggregate-forming lipophilic cation JC-1.Q37494492
Amyloid in neurodegenerative diseases: friend or foe?Q37860535
The ubiquitin proteasome system in neurodegenerative diseases: culprit, accomplice or victim?Q37978123
Review: quantifying mitochondrial dysfunction in complex diseases of aging.Q37998247
Gene expression profile of aging in human muscleQ38353969
A global test for groups of genes: testing association with a clinical outcomeQ38522319
Modeling oculopharyngeal muscular dystrophy in myotube cultures reveals reduced accumulation of soluble mutant PABPN1 proteinQ38575523
PABPN1 shuts down alternative poly(A) sitesQ39340896
The poly(A)-binding protein nuclear 1 suppresses alternative cleavage and polyadenylation sites.Q39364902
Reversible aggregation of PABPN1 pre-inclusion structuresQ39494752
Cellular senescence in human myoblasts is overcome by human telomerase reverse transcriptase and cyclin-dependent kinase 4: consequences in aging muscle and therapeutic strategies for muscular dystrophiesQ40121913
Poly(A) tail length is controlled by the nuclear poly(A)-binding protein regulating the interaction between poly(A) polymerase and the cleavage and polyadenylation specificity factorQ41806905
Deregulation of the ubiquitin-proteasome system is the predominant molecular pathology in OPMD animal models and patientsQ42868694
Sirtuin inhibition protects from the polyalanine muscular dystrophy protein PABPN1.Q46656918
Human muscle aging: ROS-mediated alterations in rectus abdominis and vastus lateralis muscles.Q46742968
Wild-type PABPN1 is anti-apoptotic and reduces toxicity of the oculopharyngeal muscular dystrophy mutation.Q46820379
Oculopharyngeal muscular dystrophy with limb girdle weakness as major complaintQ47348943
Skeletal muscle gene expression profiles in 20-29 year old and 65-71 year old womenQ47908413
Changes in lamina structure are followed by spatial reorganization of heterochromatic regions in caspase-8-activated human mesenchymal stem cells.Q50714075
Extracting biological meaning from large gene lists with DAVID.Q51795802
Expression of the polyalanine expansion mutant of nuclear poly(A)-binding protein induces apoptosis via the p53 pathway.Q53173112
Prevention of oculopharyngeal muscular dystrophy-associated aggregation of nuclear poly(A)-binding protein with a single-domain intracellular antibodyQ60297063
Nuclear inclusions in oculopharyngeal dystrophyQ72843808
P4510describes a project that useslimmaQ112236343
P433issue6
P921main subjectmuscle weaknessQ270421
P304page(s)412-426
P577publication date2013-06-01
P1433published inAgingQ2845875
P1476titleA decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle aging
P478volume5

Reverse relations

cites work (P2860)
Q85822831Ageing and muscular dystrophy differentially affect murine pharyngeal muscles in a region-dependent manner
Q42243485An alanine expanded PABPN1 causes increased utilization of intronic polyadenylation sites
Q40570752Blood RNA expression profiles undergo major changes during the seventh decade
Q37428337Control of mRNA stability contributes to low levels of nuclear poly(A) binding protein 1 (PABPN1) in skeletal muscle
Q40629006Cytokine genes as potential biomarkers for muscle weakness in OPMD.
Q100737078Cytoskeletal disorganization underlies PABPN1-mediated myogenic disability
Q64236077Deacetylation Inhibition Reverses PABPN1-Dependent Muscle Wasting
Q89146104Deregulation of RNA Metabolism in Microsatellite Expansion Diseases
Q64895891Diagnostics of short tandem repeat expansion variants using massively parallel sequencing and componential tools.
Q42907623Dysfunctional transcripts are formed by alternative polyadenylation in OPMD.
Q48112558Functional impact of an oculopharyngeal muscular dystrophy mutation in PABPN1.
Q91843569Inhibition of myostatin improves muscle atrophy in oculopharyngeal muscular dystrophy (OPMD)
Q35093811Major aging-associated RNA expressions change at two distinct age-positions.
Q90733720Mouse models for muscular dystrophies: an overview
Q50962265Novel mouse models of oculopharyngeal muscular dystrophy (OPMD) reveal early onset mitochondrial defects and suggest loss of PABPN1 may contribute to pathology.
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.
Q88004256Post-transcriptional regulation of gene expression and human disease
Q45860522Proteasomal activity-based probes mark protein homeostasis in muscles
Q38876359Regulated Intron Retention and Nuclear Pre-mRNA Decay Contribute to PABPN1 Autoregulation

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