Skip to main content
. 2015 Aug 19;25(6):663–678. doi: 10.1111/bpa.12290

Figure 3.

figure

Schematic overview of cerebellar and brainstem pathologies in PBD in men and mice. PBD are caused by mutations in proteins involved in peroxisomal matrix protein import and peroxisomal membrane protein (PMP) import. PMPs contain a C‐terminal (PTS1) or a N‐terminal (PTS2) peroxisomal targeting signal. PTS1 matrix proteins are recognized by a shorter variant of the peroxisomal import receptor PEX5 (5S). The PTS1/PEX5 complex binds to the docking complex at the peroxisomal membrane, which leads to import of the protein in the peroxisomal lumen. PTS2 proteins are imported in a similar way, but are first recognized by PEX7 that binds to the longer variant of PEX5. Peroxisomal membrane proteins are incorporated via a mechanism involving PEX19, PEX16 and PEX3.