Figure 6

Model of protein and mRNA quality control mechanisms identified in P23H rhodopsin interactome. On the left, wild-type rhodopsin (blue) is translated, folded, and processed at the ER, and transported anterograde to the rod outer segment. On the right, (1) P23H rhodopsin mRNA and nascent P23H rhodopsin protein are targeted for degradation through co-translational quality control mechanisms such as ribosome-quality control and mRNA decay. (2) fully synthesized P23H rhodopsin protein (that escapes quality control steps during translation) is targeted for degradation through post-translational quality control mechanisms such as ERAD. ERAD retrotranslocates fully synthesized P23H rhodopsin from ER to cytosol for ubiquitination and proteasomal degradation. (3) Any remaining P23H rhodopsin protein that escapes translational and post-translational quality control steps progresses to outer segment and abnormally accumulates in synapse.