Xenbase Image ID: 140135

Figure 8. Schematic summarizing the relationship between peripherin-2 subunit assembly, targeting and ADRP. WT (white) and the P216L (shaded) peripherin-2, which form a mixture of core tetramers and disulfide-linked oligomers in association with endogenous X. laevis peripherin-2, target normally to ROS disks (patterned region). The P216L mutant causes ADRP through a dominant negative mechanism. The C150S mutant, which forms core tetramers but not disulfide-linked oligomers, also targets to ROS disks. The L185P mutant forms homodimers and disulfide-linked tetramers, which are retained in the inner segment, and noncovalent tetramers and oligomers with WT peripherin-2, which target to ROS. The C214S mutant forms homodimers, disulfide-linked tetramers and aggregates. These complexes are retained in the cell body, inner segments and cilium. The C214S and L185P mutants cause ADRP through a deficiency in functional core tetramers. Image published in: Loewen CJ et al. (2003) Copyright © 2003. Image reproduced with permission of the Publisher, American Society for Cell Biology (ASCB). This is an Open Access article. Larger Image Printer Friendly View

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