Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy

BMC Neurology

Volumn 13, Issue , 2013, Pages

  Raz, Vered ^a   Sterrenburg, Ellen ^a   Routledge, Samantha ^b   Venema, Andrea ^a   van der Sluijs, Barbara M ^c   Trollet, Capucine ^d,e   Dickson, George ^d   van Engelen, Baziel G M ^c   van der Maarel, Silvère M ^a   Antoniou, Michael N ^b  

^a LEIDEN UNIVERSITY MEDICAL CENTER   (Netherlands)

^b GUY S HOSPITAL   (United Kingdom)

^c RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

^d UNIVERSITY OF LONDON   (United Kingdom)

^e University of Versailles Saint Quentin en Yvelines   (France)

Author keywords

Extracellular matrix; Muscle fibrosis; OPMD; PABPN1; PCOLCE

Indexed keywords

C ENDOPEPTIDASE ENHANCER 1 PROTEIN; MESSENGER RNA; POLY(A) BINDING PROTEIN NUCLEAR 1; POLYADENYLIC ACID BINDING PROTEIN; PROCOLLAGEN C PROTEINASE; UNCLASSIFIED DRUG;

ADULT; AGED; ANIMAL CELL; ARTICLE; CELL FUSION; CELLULAR DISTRIBUTION; CLINICAL ARTICLE; CONTROLLED STUDY; DOWN REGULATION; EXTRACELLULAR MATRIX; FEMALE; HUMAN; HUMAN CELL; IN VITRO STUDY; MALE; MOUSE; MUSCLE ATROPHY; MUSCLE BIOPSY; MUSCLE CELL; MYOBLAST; MYOTUBE; NONHUMAN; OCULOPHARYNGEAL MUSCULAR DYSTROPHY; PROTEIN AGGREGATION; PROTEIN DEPLETION; PROTEIN EXPRESSION; TIBIALIS ANTERIOR MUSCLE;

AGE FACTORS; ALANINE; ANIMALS; CELL NUCLEOLUS; CELLS, CULTURED; COLLAGEN; DISEASE MODELS, ANIMAL; DOWN-REGULATION; EXTRACELLULAR MATRIX PROTEINS; GLYCOPROTEINS; HUMANS; IMMUNOPRECIPITATION; MICE; MICE, KNOCKOUT; MUSCLE PROTEINS; MUSCLE, SKELETAL; MUSCULAR DYSTROPHY, OCULOPHARYNGEAL; MYOBLASTS; POLY(A)-BINDING PROTEIN I; TRANSFECTION;

EID: 84879496543     PISSN: None     EISSN: 14712377     Source Type: Journal    
DOI: 10.1186/1471-2377-13-70     Document Type: Article

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