Figure 2.

Immunofluorescence analysis of primary human fibroblasts with anti-catalase antibody. (a) In normal human fibroblasts, the peroxisomal matrix protein catalase is localized in the peroxisome as indicated by the punctate pattern. (b) ZSS patient fibroblasts fail to import catalase correctly into the peroxisome and thus show diffused localization of catalase in the cytosol. (c) In rare cases, ZSS patient fibroblasts show mosaicism, in which catalase is localized in the peroxisome as well as in the cytosol.