Kamp, E.H. van de

glucosidase enzyme muscle glycogen disease tissue knockout heart activity knockout mice mouse storage lysosomal gsdii glucosidase activity enzyme replacement therapy model rhgaa rabbit genetic patient reuser figure liver deficiency therapy injection ko mice wild-type 6 weeks knockout mouse glycogen storage placental acid section replacement a.j.j table microscopy electron treatment study electron microscopy antibody knockout mouse model c 57bl mice 13 weeks glycogen content glucosidase deficiency lysosome animal pompe cassette acid maltase deficiency analysis heart muscle gaa gene protein mouse model lysosomal glycogen storage glycogenosi quail fibre production effect lysosomal glycogen enzyme administration +++ femoral muscle muscle fibres content result animal models primer acid phosphatase gsdii knockout mice glycogen storage disease ploeg 68 mg /kg sequence c 57bl background