A beta-blocker, propranolol, decreases the efficacy from enzyme replacement therapy in Pompe disease
Enzyme replacement therapy (ERT) with recombinant human acid α-glucosidase (rhGAA) fails to completely reverse muscle weakness in Pompe disease. β2-agonists enhanced ERT by increasing receptor-mediated uptake of rhGAA in skeletal muscles. To test the hypothesis that a β-blocker might reduce the effi...
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Veröffentlicht in: | Molecular genetics and metabolism 2016-02, Vol.117 (2), p.114-119 |
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Format: | Artikel |
Sprache: | eng |
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Zusammenfassung: | Enzyme replacement therapy (ERT) with recombinant human acid α-glucosidase (rhGAA) fails to completely reverse muscle weakness in Pompe disease. β2-agonists enhanced ERT by increasing receptor-mediated uptake of rhGAA in skeletal muscles.
To test the hypothesis that a β-blocker might reduce the efficacy of ERT, because the action of β-blockers opposes those of β2-agonists.
Mice with Pompe disease were treated with propranolol (a β-blocker) or clenbuterol in combination with ERT, or with ERT alone.
Propranolol-treated mice had decreased weight gain (p |
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ISSN: | 1096-7192 1096-7206 |
DOI: | 10.1016/j.ymgme.2015.09.012 |