Modeling oculopharyngeal muscular dystrophy in myotube cultures reveals reduced accumulation of soluble mutant pabpn1 protein

American Journal of Pathology

Volumn 179, Issue 4, 2011, Pages 1988-2000

  Raz, Vered ^a   Routledge, Samantha ^b   Venema, Andrea ^a   Buijze, Hellen ^a   Van Der Wal, Erik ^a   Anvar, Seyedyahya ^a   Straasheijm, Kirsten R ^a   Klooster, Rinse ^a   Antoniou, Michael ^b   Van Der Maarel, Silvre M ^a  

^a LEIDEN UNIVERSITY MEDICAL CENTER   (Netherlands)

^b GUY S HOSPITAL   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

MUTANT PROTEIN; POLY(A) BINDING PROTEIN NUCLEAR 1; PROTEASOME; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL DIFFERENTIATION; COMPARATIVE STUDY; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME INHIBITION; GENE EXPRESSION; HUMAN; MOUSE; MUSCLE WEAKNESS; MYOBLAST; MYOTUBE; NONHUMAN; OCULOPHARYNGEAL MUSCULAR DYSTROPHY; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN METABOLISM; QUANTITATIVE ANALYSIS; TRANSGENE;

ANIMALS; BASE SEQUENCE; CELLS, CULTURED; DESMIN; DISEASE MODELS, ANIMAL; HUMANS; INTRANUCLEAR INCLUSION BODIES; MICE; MOLECULAR SEQUENCE DATA; MUSCLE FIBERS, SKELETAL; MUSCLES; MUSCULAR DYSTROPHY, OCULOPHARYNGEAL; MUTANT PROTEINS; POLY(A)-BINDING PROTEIN II; POLYUBIQUITIN; PROTEASOME ENDOPEPTIDASE COMPLEX; PROTEIN STRUCTURE, QUATERNARY; SIGNAL TRANSDUCTION; SOLUBILITY; TRANSCRIPTOME; TRANSFECTION; TRINUCLEOTIDE REPEAT EXPANSION; UBIQUITINATION;

EID: 80053231188     PISSN: 00029440     EISSN: 15252191     Source Type: Journal    
DOI: 10.1016/j.ajpath.2011.06.044     Document Type: Article

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