Zebrafish models of BAG3 myofibrillar myopathy suggest a toxic gain of function leading to BAG3 insufficiency

Acta Neuropathologica

Volumn 128, Issue 6, 2014, Pages 821-833

  Ruparelia, Avnika A ^a   Oorschot, Viola ^a   Vaz, Raquel ^a   Ramm, Georg ^a   Bryson Richardson, Robert J ^a  

^a MONASH UNIVERSITY   (Australia)

Author keywords

Autophagy; BAG3; Muscle; Myofibrillar myopathy; Zebrafish

Indexed keywords

ALPHA ACTININ; BCL2 ASSOCIATED ATHANOGENE 3 PROTEIN; DYSTROPHIN; MUTANT PROTEIN; MYOSIN; PROTEIN; PROTEIN AGGREGATE; UNCLASSIFIED DRUG; APOPTOSIS REGULATORY PROTEIN; BAG3 PROTEIN, HUMAN; BAG3 PROTEIN, ZEBRAFISH; SIGNAL TRANSDUCING ADAPTOR PROTEIN; ZEBRAFISH PROTEIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; AUTOPHAGY; BLEACHING; CONTROLLED STUDY; EMBRYO; FAST MUSCLE FIBER; IMMUNOHISTOCHEMISTRY; IN SITU HYBRIDIZATION; MISSENSE MUTATION; MYOFIBRILLAR MYOPATHY; MYOPATHY; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN EXPRESSION; PROTEIN INTERACTION; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; SLOW MUSCLE FIBER; TEMPERATURE; ZEBRA FISH; ANIMAL; CYTOPLASM; DISEASE MODEL; DRUG EFFECTS; GENE SILENCING; GENETICS; HEART; HUMAN; METABOLISM; MUSCLE CONTRACTION; MUSCLE FIBRIL; MUTATION; PATHOPHYSIOLOGY; PHYSIOLOGY; SARCOMERE; SKELETAL MUSCLE; TRANSGENIC ANIMAL;

ADAPTOR PROTEINS, SIGNAL TRANSDUCING; ANIMALS; ANIMALS, GENETICALLY MODIFIED; APOPTOSIS REGULATORY PROTEINS; AUTOPHAGY; CYTOPLASM; DISEASE MODELS, ANIMAL; GENE KNOCKDOWN TECHNIQUES; HEART; HUMANS; MUSCLE CONTRACTION; MUSCLE, SKELETAL; MUTATION; MYOFIBRILS; MYOPATHIES, STRUCTURAL, CONGENITAL; PROTEIN AGGREGATES; SARCOMERES; TEMPERATURE; ZEBRAFISH; ZEBRAFISH PROTEINS;

EID: 84921936490     PISSN: 00016322     EISSN: 14320533     Source Type: Journal    
DOI: 10.1007/s00401-014-1344-5     Document Type: Article

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