Figure 3.

SGSH Uptake by MPS IIIA Patient Fibroblasts

(A) Conditioned media containing either WT SGSH or SGSHv4, plus or minus M6P, were applied to MPS IIIA patient fibroblasts and enzyme activity in cell lysates assessed 6 hr later. Data represent mean ± SD. (B) Immunohistochemistry for human SGSH after application of conditioned media to MPS IIIA fibroblasts. Conditioned media as in (A) was applied for 6 hr, after which the cells were fixed, stained for human SGSH, and imaged. Insets are enlargements of sites marked with an asterisk (*). Scale bar, 25 μm. (C) Conditioned media containing SGSH or SGSHv4 were applied to MPS IIIA patient fibroblasts for 6 hr, and SGSH protein levels in cell lysates were analyzed by western blot. For all panels, n = 3 biological replicates, in triplicate.