UNASSIGNED: Enzyme replacement therapy (ERT) with recombinant @entity1 acid a-glucosidase (rhGAA) fails to completely reverse @entity425 in @entity13785 . b2-agonists enhanced ERT by increasing receptor-mediated uptake of rhGAA in skeletal muscles. PURPOSE: To test the hypothesis that @entity15370 -blocker might reduce the efficacy of ERT, because the action of b-blockers opposes those of b2-agonists. METHODS: @entity19 with @entity13785 were treated with @entity1169 ( @entity15370 -blocker) or @entity16959 in combination with ERT, or with ERT alone. RESULTS: @entity1169 -treated @entity19 had decreased @entity1017 (p<0.01), in comparison with @entity16959 -treated @entity19 . Left ventricular mass was decreased (and comparable to wild-type) in ERT only and @entity16959 -treated groups of @entity19 , and unchanged in @entity1169 -treated @entity19 . GAA activity increased following either @entity16959 or @entity1169 in skeletal muscles. However, muscle glycogen was reduced only in @entity16959 -treated @entity19 , not in @entity1169 -treated @entity19 . Cell-based experiments confirmed that @entity1169 reduces uptake of rhGAA into Pompe fibroblasts and also demonstrated that the drug induces intracellular accumulation of glycoproteins at higher doses. CONCLUSION: @entity1169 , a commonly prescribed b-blocker, reduced weight, increased left ventricular mass and decreased glycogen clearance in skeletal muscle following ERT. b-Blockers might therefore decrease the efficacy from ERT in @entity1 with @entity13785 .

@entity15370 -blocker, XXXX , decreases the efficacy from enzyme replacement therapy in @entity13785 .

@entity1 :: ('9606', 'Species') :: ['human', 'patients'] @entity15370 :: ('11820(Tax:10090)', 'Gene') :: ['a b'] @entity1169 :: ('MESH:D011433', 'Chemical') :: ['propranolol', 'Propranolol'] @entity19 :: ('10090', 'Species') :: ['mice', 'Mice'] @entity16959 :: ('MESH:D002976', 'Chemical') :: ['clenbuterol'] @entity425 :: ('MESH:D018908', 'Disease') :: ['muscle weakness'] @entity13785 :: ('MESH:D006009', 'Disease') :: ['Pompe disease'] @entity1017 :: ('MESH:D015430', 'Disease') :: ['weight gain']
@entity1169 :: (MESH:D011433,Chemical) :: ['propranolol']
