Boer, H.A. de

acid α- glucosidase glucosidase enzyme mouse expression cho cells gland lysosomal transgene activity production precursor fraction gsdii disease protein tissue fibroblast 76 kda 1-casein muscle storage medium study glycogen sample natl acad cho cell medium enzyme replacement therapy figure mouse milk number reuser level fragment animal m 6p receptor blot analysis 110 kda precursor gsdii ko mice 4- methylumbelliferyl -α-d fraction i enzyme patient therapy probe hopwood heart biochem genetic table receptor antibody institute sequence replacement a.j.j analysis mouse lines fraction i promoter copy number transgene expression downloaded sds –page anson march epithelial cells model gsdii knockout mice glycogenosis type ii concentration rabbit digestion result deficiency knockout placenta lysosomal glycogen storage source lysosomal storage diseases