Figure 5.

Histological evidence of improved MPS I pathology after hiNSC transplantation in Idua^−/− mice

Representative immunofluorescence images of cortex and pons brain tissue show elevated expression of common neuropathic markers LAMP1 (A) and CD68 (B) in Idua^−/− mice compared to unaffected carriers (Idua^+/−). After successful engraftment of transplanted hiNSCs in Idua^−/− mice (red), levels of LAMP1 (A) and CD68 (B) (green) are similar to those observed in unaffected carriers. Conversely, in regions devoid of engraftment (absence of red), levels of LAMP1 (A) and CD68 (B) (green) are comparable to those observed in untreated Idua^−/− mice. No staining for STEM121 is observed in the first three rows because animals did not receive hiNSCs. Scale bars, 50 μm.