Normalized histogram overlays of wild type fibroblast
(WT, A) and
MPS IVA patient’s fibroblast (GM00593, B, and GM01361, C) untreated
and treated with BC. (D) Fold change in MFI relative to the control
group, labeled WT fibroblast, was calculated. Three independent experiments
per BC treatment and cell source were analyzed via flow cytometry
(***, p < 0.001).