Retinal degeneration 6 rd6

Retinal degeneration 6 (rd6)

Ophthalmoscopic examination of mice homozygous for rd6 reveals discrete sub-retinal spots oriented in a regular pattern across the retina. The retinal spots appear by 8 – 10 weeks of age and persist through advanced stages of retinal degeneration. Histologic examination reveals large cells in the sub-retinal space, typically juxtaposed to the retinal pigment epithelium. The white dots seen on fundus examination correspond both in distribution and size to these large cells. By three months of age, the cells are filled with membranous profiles, lipofuscin-like material and pigment. These cells react strongly with an antibody directed against a mouse macrophage-associated antigen. Photoreceptor cells progressively degenerate with age, and an abnormal electroretinogram is initially detected between one and two months of age.