Adjunctive β2-agonist treatment reduces glycogen independently of receptor-mediated acid α-glucosidase uptake in the limb muscles of mice with Pompe disease

FASEB Journal

Volumn 28, Issue 5, 2014, Pages 2272-2280

  Farah, Benjamin L ^a   Madden, Lauran ^b   Li, Songtao ^b   Nance, Sierra ^b,c   Bird, Andrew ^b   Bursac, Nenad ^b   Yen, Paul M ^a,b   Young, Sarah P ^b   Koeberl, Dwight D ^b  

^a DUKE NUS MEDICAL SCHOOL   (Singapore)

^b DUKE UNIVERSITY MEDICAL CENTER   (United States)

^c WINSTON SALEM STATE UNIVERSITY   (United States)

Author keywords

Acid maltase; Adenoassociated virus; Autophagy; Enzyme replacement therapy; Gene therapy; Mannose 6 phosphate receptor

Indexed keywords

ADENOVIRUS VECTOR; ALPHA GLUCOSIDASE; CLENBUTEROL; GLYCOGEN; LYSOSOME ASSOCIATED MEMBRANE PROTEIN 2; SOMATOMEDIN B RECEPTOR; TETRASACCHARIDE;

ADENO ASSOCIATED VIRUS; ADJUVANT THERAPY; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; AUTOPHAGOSOME; CELL MIGRATION; CELL VACUOLE; CONTROLLED STUDY; DIAPHRAGM MUSCLE; DRUG EFFICACY; ENZYME REPLACEMENT; GENE INACTIVATION; GLUCOSE URINE LEVEL; GLYCOGEN ANALYSIS; GLYCOGEN MUSCLE LEVEL; GLYCOGEN STORAGE DISEASE TYPE 2; IMMUNOHISTOCHEMISTRY; IN VIVO STUDY; LYSOSOME; MACROAUTOPHAGY; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; QUADRICEPS FEMORIS MUSCLE; ROTAROD TEST; SKELETAL MUSCLE; SOLEUS MUSCLE;

ACID MALTASE; ADENO-ASSOCIATED VIRUS; AUTOPHAGY; ENZYME REPLACEMENT THERAPY; GENE THERAPY; MANNOSE-6-PHOSPHATE RECEPTOR;

ADRENERGIC BETA-2 RECEPTOR AGONISTS; ALPHA-GLUCOSIDASES; ANIMALS; CATIONS; CLENBUTEROL; DENSITOMETRY; DEPENDOVIRUS; EXTREMITIES; GENETIC VECTORS; GLYCOGEN; GLYCOGEN STORAGE DISEASE TYPE II; HEK293 CELLS; HUMANS; LYSOSOMES; MICE; MICE, KNOCKOUT; MUSCLE, SKELETAL; RECEPTOR, IGF TYPE 2;

EID: 84901069861     PISSN: 08926638     EISSN: 15306860     Source Type: Journal    
DOI: 10.1096/fj.13-244202     Document Type: Article

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