Chemical chaperone ameliorates pathological protein aggregation in plectin-deficient muscle

Journal of Clinical Investigation

Volumn 124, Issue 3, 2014, Pages 1144-1157

  Winter, Lilli ^a   Staszewska, Ilona ^a   Mihailovska, Eva ^a   Fischer, Irmgard ^a   Goldmann, Wolfgang H ^b   Schröder, Rolf ^c   Wiche, Gerhard ^a  

^a UNIVERSITY OF VIENNA   (Austria)

^b UNIVERSITY OF ERLANGEN NUREMBERG   (Germany)

^c UNIVERSITY HOSPITAL ERLANGEN   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA ACTININ; ALPHA CRYSTALLIN; CHAPERONE; DESMIN; HEAT SHOCK PROTEIN 27; HEAT SHOCK PROTEIN 70; HEAT SHOCK PROTEIN 90; PLECTIN; PROTEIN P53;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CELL DIFFERENTIATION; CELLULAR DISTRIBUTION; CONTROLLED STUDY; EXTENSOR DIGITORUM LONGUS MUSCLE; FLUORESCENCE RECOVERY AFTER PHOTOBLEACHING; IMMUNOBLOTTING; IMMUNOFLUORESCENCE MICROSCOPY; IMMUNOHISTOCHEMISTRY; INTERMEDIATE FILAMENT; MITOCHONDRION; MOUSE; MUSCLE DISEASE; MYOBLAST; MYOTUBE; NONHUMAN; PATHOGENESIS; PHENOTYPE; PLECTINOPATHY; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN CONFORMATION; PROTEIN HOMEOSTASIS; PROTEIN LOCALIZATION; SARCOMERE; UPREGULATION;

ANIMALS; CELL DIFFERENTIATION; CELLS, CULTURED; DESMIN; DRUG EVALUATION, PRECLINICAL; HEAT-SHOCK PROTEINS; INTERMEDIATE FILAMENTS; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MUSCLE FIBERS, SKELETAL; MUSCLE STRENGTH; MUSCLE, SKELETAL; MYOBLASTS; MYOPATHIES, STRUCTURAL, CONGENITAL; PHENYLBUTYRATES; PLECTIN; PROTEIN STABILITY; SARCOMERES; UP-REGULATION;

EID: 84896773754     PISSN: 00219738     EISSN: 15588238     Source Type: Journal    
DOI: 10.1172/JCI71919     Document Type: Article

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