[HTML][HTML] Probing Mechanisms of Photoreceptor Degeneration in a New Mouse Model of the Common Form of Autosomal Dominant Retinitis Pigmentosa due to P23H …

S Sakami, T Maeda, G Bereta, K Okano… - Journal of Biological …, 2011 - ASBMB
Rhodopsin, the visual pigment mediating vision under dim light, is composed of the
apoprotein opsin and the chromophore ligand 11-cis-retinal. A P23H mutation in the opsin
gene is one of the most prevalent causes of the human blinding disease, autosomal
dominant retinitis pigmentosa. Although P23H cultured cell and transgenic animal models
have been developed, there remains controversy over whether they fully mimic the human
phenotype; and the exact mechanism by which this mutation leads to photoreceptor cell …