Phenotype Image Detail

Image

Caption

Nine month old Cp^tm1Hrs/Cp^tm1Hrs Heph^sla/Y (Cp-/-Heph-/Y) mice have retinal degeneration. A: Light photomicrograph of wild-type retina. B & C: Cp^tm1Hrs/Cp^tm1Hrs Heph^sla/Y retina has focal patches of hypertrophic retinal pigment epithelium (RPE) cells in some areas (B) and confluent hypertrophic RPE cells in other areas (C). D: In an area of RPE hyperplasia (demarcated by arrowheads), double mutant retinas have local photoreceptor degeneration [demarcated by arrows in the outer nuclear layer (ONL)] and subretinal neovascularization (red *). E: In an area of hypertrophic, hyperplastic (area demarcated by arrowheads) RPE cells, a necrotic RPE cell also observed by electron microscopy (Left Inset) is present. Within the area of RPE hyperplasia, there is local photoreceptor thinning and subretinal neovascularization (red *) visible as small vessels containing erythrocytes (Right Inset). The hyperplastic RPE have formed a localized cyst (Cy). F: Electron micrograph of wild-type RPE. Br, Bruch's membrane; AM, apical microvilli; OS, photoreceptor outer segments. G: Electron micrograph of double mutant mouse RPE overloaded with phagosomes and lysosomes containing photoreceptor outer segments at various stages of digestion. Some of these lysosomes (*) contained multilamellar structures characteristic of outer segment membranes (Inset). H: Electron micrograph of double mutant mouse deposits between RPE and Bruch's membrane containing wide-spread collagen (*). Scale bars: A-E, 50um; F & G, 2um; H, 500nm

Copyright

This image is from Hahn P, Proc Natl Acad Sci U S A 2004 Sep 21;101(38):13850-5. Copyright 2004 National Academy of Sciences, U.S.A. J:92620

Associated Alleles

Symbol	Name
Cp^tm1Hrs	ceruloplasmin; targeted mutation 1, Z Leah Harris
Heph^sla	hephaestin; sex-linked anemia

Associated Genotypes

Allelic Composition	Genetic Background
Cp^tm1Hrs/Cp^tm1Hrs Heph^sla/Y	involves: 129X1/SvJ * C57BL/6