Smiley, Sheila2014-07-162014-07-1620142014http://hdl.handle.net/10393/31330http://dx.doi.org/10.20381/ruor-3832Human vision is heavily dependent on cone photoreceptor function and irreversible loss of these cells, caused by retinal degenerative diseases, results in deterioration of central, high acuity vision. Here, I characterized coiled-coil domain containing 136 (Ccdc136) as a cone marker. Ccdc136-/- mice were generated and examined using immunohistochemical and electrophysiological analyses. To investigate cone transplantation, the mice were crossed onto a cone-only neural retina leucine zipper knockout (Nrl-/-) background generating Nrl-/-;Ccdc136+/- donor mice. Ccdc136-/- mice expressed GFP in cone but not rod photoreceptors. Nrl-/-;Ccdc136+/- donor mice had an enriched population of GFP+ cones and showed enhanced cone function, identical to Nrl- /- mice. After transplantation, Nrl-/-;Ccdc136+/- cells migrated successfully into adult recipient retinas. Morphology of integrated cells improved with younger donor age with some integrated cells expressing cone markers. In conclusion, the Nrl-/-;Ccdc136+/- mouse is an appropriate strain to investigate cone photoreceptor transplantation. Future studies aim to improve integration rates to assess vision rescue in degenerated retinas.enThesis