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Figure 4 | BMC Medical Genetics

Figure 4

From: A founder mutation in the PEX6 gene is responsible for increased incidence of Zellweger syndrome in a French Canadian population

Figure 4

Decreased peroxisome number and matrix protein import in cultured fibroblasts. Immunofluorescence-based peroxisome assembly assays were performed on cultured skin fibroblasts obtained from ZS patient B1 and the same PEX6 null cell line used in Figure 3 (Panels a-d) and healthy control (Panels e and f). Briefly, cells were fixed, permeabilized and stained with anti-human PMP70 (secondary, texas red) and thiolase (secondary FITC) antiserum to highlight peroxisome membrane and matrix proteins, respectively

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