Eric Merrell

Bound Volume Number

Volume II

Degree Type

Honors Capstone Project

Date of Submission

Spring 5-2016

Capstone Advisor

Barry Knox

Capstone Major


Capstone College

Arts and Science

Audio/Visual Component



Misfolded Protein Degradation, Retinitis Pigmentosa, Rhodopsin-P23H Mutation

Capstone Prize Winner


Won Capstone Funding


Honors Categories

Sciences and Engineering

Subject Categories



Retinitis pigmentosa (RP) is a term used to describe a wide variety of inherited degenerative diseases that affect the eye. While there are many causes of this disease, the most commonly found mutation that causes RP in North America is an autosomal dominant missense mutation in rhodopsin (RhoP23H). Previous studies have shown that RhoP23H is predominantly misfolded, resulting in a dramatic loss of the ability to stably bind 11-cis retinal and thus function as a photopigment. Previous work has shown that this process is conserved to some degree across many models, from pigs to mice, and even is evident when mutant mammalian rhodopsin is exogenously expressed in flies. Presently, there is limited information on the mechanism(s) that detect and degrade rhodopsin. To investigate this phenomenon, we cultured transgenic Drosophila melanogaster and evaluated expression of exogenous rhodopsin through western blot analysis. Our results suggest that the fly system is capable of simulating a realistic environment for murine rhodopsin. By harnessing the powerful genetic tools surrounding the fly system, future studies using RNAi techniques may be able to elucidate identification and degradation pathways important to the progression of RP.

Creative Commons License

Creative Commons Attribution 3.0 License
This work is licensed under a Creative Commons Attribution 3.0 License.

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