New experimental model for RP – Schapendoe hunting dogs point the way

Researchers from Ruhr-University in Bochum, Germany, have reported the generation of a new murine model of retinitis pigmentosa (RP). The development of the new model follows on from previous studies by the same research group showing an association between the coiled coil domain-containing 66 gene (“Ccdc66”) and the canine equivalent of RP, “generalised progressive retinal atrophy” (gPRA). The original observations, made by Drs. Wanda M. Gerding and Jorg T. Epplen in Schapendoes dogs, a Dutch breed of herding dog, have advanced rapidly to characterise the pathology underlying inactivation of Ccdc66. The report, in Human Molecular Genetics (2011, doi:10.1093/hmg/ddr282), now provides a new murine model which may more accurately reflect the time course of retinal degeneration seen in RP patients.