Antiprion drugs 6-aminophenanthridine and guanabenz reduce PABPN1 toxicity and aggregation in oculopharyngeal muscular dystrophy

EMBO Molecular Medicine

Volumn 3, Issue 1, 2011, Pages 35-49

  Barbezier, Nicolas ^a   Chartier, Aymeric ^a   Bidet, Yannick ^a   Buttstedt, Anja ^b   Voisset, Cécile ^c   Galons, Hervé ^d   Blondel, Marc ^c   Schwarz, Elisabeth ^b   Simonelig, Martine ^a  

^a INSTITUTE OF HUMAN GENETICS   (France)

^b MARTIN LUTHER UNIVERSITY HALLE WITTENBERG   (Germany)

^c FACULTÉ DE MÉDECINE ET DES SCIENCES DE LA SANTÉ   (France)

^d UNIVERSITÉ PARIS DESCARTES   (France)

Author keywords

Anti aggregation drug; Drosophila model; Muscular dystrophy; PABPN1; Ribosomal RNA

Indexed keywords

6 AMINOPHENANTHRIDINE; AMYLOID; GUANABENZ ACETATE; PHENANTHRIDINE DERIVATIVE; POLYADENYLIC ACID BINDING PROTEIN; PRION PROTEIN; RIBOSOME DNA; RIBOSOME RNA; UNCLASSIFIED DRUG;

AMINO TERMINAL SEQUENCE; ANIMAL MODEL; ARTICLE; CELL NUCLEUS INCLUSION BODY; DROSOPHILA; DRUG EFFICACY; DRUG STRUCTURE; MUSCLE ATROPHY; NONHUMAN; OCULOPHARYNGEAL MUSCULAR DYSTROPHY; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN FOLDING;

ANIMALS; DROSOPHILA; GUANABENZ; LARVA; MUSCULAR DYSTROPHY, OCULOPHARYNGEAL; PHENANTHRIDINES; PHENOTYPE; POLY(A)-BINDING PROTEIN II; PRION DISEASES; PROTEIN FOLDING; RNA, RIBOSOMAL;

EID: 78650822020     PISSN: 17574676     EISSN: 17574684     Source Type: Journal    
DOI: 10.1002/emmm.201000109     Document Type: Article

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