Figure 5.

GNeo delivery of lysosomal enzymes enhances turnover of glycosaminoglycans in mucopolysaccharidosis (MPS) fibroblasts. (a,b) Normal and MPS VII or (c) MPS I fibroblasts were radiolabeled with ³⁵SO₄ and chased for 24 hours with the indicated concentration of bovine β-glucuronidases (a), human β-glucuronidases (b), and human α--iduronidase (c). The amount of [³⁵S]glycosaminoglycan remaining was measured (Materials and Methods). (a) GUS (squares), AP-GUS (triangles), or GNeo-AP-GUS (filled circles). (b) hGUS (squares), AP-hGUS (triangles), or GNeo-AP-GUS (filled circles). (c) Iduronidase (squares), AP-iduronidase (triangles), or GNeo-AP-iduronidase (filled circles). The dotted line represents the amount of [³⁵S]glycosaminoglycan remaining in normal fibroblasts (HFF) without enzyme supplementation. The experiment was performed twice, in triplicate.