![]() ![]() ![]() Usually, patients first suffer from peripheral visual field loss because of peripheral rod photoreceptor cell death. RP affects 19–27 out of 100,000 people all over the world. Retinitis pigmentosa (RP, MIM 26800) is a common group of inherited retinal diseases that lead to blindness. In short, our study indicates that in some RP, adenylyl cyclase is involved in photoreceptor cell death pathway its inhibition is potentially a logical approach for a novel RP therapy. This fish and rd10 model mice show photoreceptor cell death caused by adenylyl cyclase. For further confirmation, we have also generated a transgenic fish which possesses a human rhodopsin mutation, Q344X. Furthermore, we have demonstrated that forced activation of adenylyl cyclase in the inner segment leads to rod photoreceptor cell death. So, ectopic phototransduction activated by light exposure, which leads to rod photoreceptor cell death, is through the action of transducin. However, knockdown of phosphodiesterase, the enzyme that mediates the next step of phototransduction, does not. The ovl larvae show ectopic accumulation of rhodopsin and knockdown of ectopic rhodopsin and transducin rescue rod photoreceptor cell death. In ovl, a zebrafish model of outer segment deficiency, photoreceptor cell death is associated with light exposure. In this study, we have shown that even a mild light exposure can induce ectopic phototransduction and result in the acceleration of rod photoreceptor cell death in some vertebrate models. However, the detailed mechanism by which photoreceptor cell death is caused by light exposure is unclear. Among them, light exposure has been reported to induce photoreceptor cell death. RP is a large family of diseases in which the photoreceptor cell death can be caused by a number of pathways. Most of inherited retinal diseases such as retinitis pigmentosa (RP) cause photoreceptor cell death resulting in blindness. ![]()
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