Figure 5. Immunostaining of tissue sections from mice after dual-ERT.

A) Numerous cells expressing both PPCA and Neu1 were detected with anti-PPCA and anti-Neu1 antibodies in liver (Li), spleen (Sp), and kidney (Ki). B) Cells expressing only PPCA were detected in adrenal gland (AG), heart (He), intestine (In), testis (Te), and choroid plexus (CP). Punctuated staining demonstrates internalization of corrective enzyme mostly by Kuppfer cells in the liver and resident macrophages in other tissues. Magnification = x400 Adapted from Bonten et al. Faseb Journal 2004, ⁵⁹ with permission of FASEB.