Membrane blebbing as an assessment of functional rescue of dysferlin-deficient human myotubes via nonsense suppression

Journal of Applied Physiology

Volumn 109, Issue 3, 2010, Pages 901-905

  Wang, Bingjing ^a   Yang, Zhaohui ^a   Brisson, Becky K ^a   Feng, Huisheng ^a   Zhang, Zhiqian ^b   Welch, Ellen M ^c   Peltz, Stuart W ^c   Barton, Elisabeth R ^a   Brown Jr , Robert H ^d   Sweeney, H Lee ^a  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b Department of Biochemistry and Molecular BiologyCancer Hospital of Peking University   (China)

^c PTC THERAPEUTICS   (United States)

^d UNIVERSITY OF MASSACHUSETTS   (United States)

Author keywords

A J mice; Ataluren; Hypotonic shock; Limb girdle muscular dystrophy type 2B; Miyoshi myopathy; Myotube cultures; PTC124

Indexed keywords

ATALUREN; DYSF PROTEIN, HUMAN; DYSF PROTEIN, MOUSE; HYPOTONIC SOLUTION; MEMBRANE PROTEIN; MUSCLE PROTEIN; OXADIAZOLE DERIVATIVE;

ANIMAL; ARTICLE; BIOASSAY; C57BL MOUSE; CELL CULTURE; CELL MEMBRANE; DRUG EFFECT; GENETIC TRANSFECTION; GENETICS; HUMAN; METABOLISM; METHODOLOGY; MOUSE; MOUSE MUTANT; NEWBORN; OSMOTIC PRESSURE; PATHOLOGY; QUADRICEPS FEMORIS MUSCLE; SKELETAL MUSCLE; STOP CODON;

ANIMALS; ANIMALS, NEWBORN; BIOLOGICAL ASSAY; CELL MEMBRANE; CELLS, CULTURED; CODON, NONSENSE; HUMANS; HYPOTONIC SOLUTIONS; MEMBRANE PROTEINS; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MUSCLE FIBERS, SKELETAL; MUSCLE PROTEINS; OSMOTIC PRESSURE; OXADIAZOLES; QUADRICEPS MUSCLE; TRANSFECTION;

EID: 77957153178     PISSN: 87507587     EISSN: 15221601     Source Type: Journal    
DOI: 10.1152/japplphysiol.01366.2009     Document Type: Article

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