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Phenotype Image Detail
Image
Caption Nine month old Cptm1Hrs/Cptm1Hrs Hephsla/Y (Cp-/-Heph-/Y) mice have retinal degeneration. A: Light photomicrograph of wild-type retina. B & C: Cptm1Hrs/Cptm1Hrs Hephsla/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
Cptm1Hrs ceruloplasmin; targeted mutation 1, Z Leah Harris
Hephsla hephaestin; sex-linked anemia
Associated
Genotypes
Allelic Composition Genetic Background
Cptm1Hrs/Cptm1Hrs
Hephsla/Y
involves: 129X1/SvJ * C57BL/6

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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
04/08/2014
MGI 5.17
The Jackson Laboratory