Cytoplasmic targeting of mutant poly(A)-binding protein nuclear 1 suppresses protein aggregation and toxicity in oculopharyngeal muscular dystrophy

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Volumn 6, Issue 9, 2005, Pages 766-779

  Abu Baker, Aida ^a   Laganiere, Simon ^a   Fan, Xueping ^a   Laganiere, Janet ^a   Brais, Bernard ^b   Rouleau, Guy A ^a  

^a MCGILL UNIVERSITY HEALTH CENTRE   (Canada)

^b NOTRE DAME HOSPITAL   (Canada)

Author keywords

Alanine expansion disorder; Intranuclear aggregates; Nuclear localization signal; Nuclear speckles; Oculopharyngeal muscular dystrophy; PML; Poly(A) binding protein nuclear 1; Protein subcellular locations

Indexed keywords

ALANINE; GREEN FLUORESCENT PROTEIN; POLYADENYLIC ACID BINDING PROTEIN; PROMYELOCYTIC LEUKEMIA PROTEIN;

AMINO TERMINAL SEQUENCE; ARTICLE; AUTOSOMAL DOMINANT INHERITANCE; CELL CULTURE; CELL NUCLEUS; CELL NUCLEUS INCLUSION BODY; CELLULAR DISTRIBUTION; CLINICAL FEATURE; CONTROLLED STUDY; CYTOPLASM; CYTOTOXICITY; DYSPHAGIA; EYELID DISEASE; GENE INSERTION; GENE MUTATION; HISTOPATHOLOGY; HUMAN; HUMAN CELL; INHIBITION KINETICS; LIMB DISEASE; MUSCLE CELL; MUSCLE WEAKNESS; NUCLEAR EXPORT SIGNAL; NUCLEAR LOCALIZATION SIGNAL; OCULOPHARYNGEAL MUSCULAR DYSTROPHY; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN DOMAIN; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; PROTEIN STRUCTURE; PROTEIN TARGETING; PROTEIN TRANSPORT; SKELETAL MUSCLE; WILD TYPE;

EID: 23844440597     PISSN: 13989219     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1600-0854.2005.00315.x     Document Type: Article

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