Inherited Retinal Degeneration

These mice harbor a point mutation in the PDE6b gene which is required for the phototransduction cascade. As such, these mice develop a severe and progressive retinal degeneration that begins with photoreceptor cell death. As opposed to other retinal degeneration models in which the retina has degenerated completely by P21, these mice do not lose their complement of photoreceptors until ~P35. Moreover, this loss can be delayed until ~P50 by rearing newborn pubs in the dark until they are of adult age (P21), which provides a larger window for therapeutic intervention. The severity of retinal degeneration can be monitored by histological cell counts, biochemical analyses of photoreceptor gene and protein expression, and visual function by electroretinography. This is an excellent animal model for screening neuroprotective compounds that could delay or prevent photoreceptor cell death that occurs in retinitis pigmentosa, macular degeneration, and diabetic retinopathy.

Preclinical Ophthalmic Contract Research