Phenotype Image Detail

Image

Caption

Tg(BEST1-HTRA1)#Ybf/0 mice with polypoidal choroidal vasculopathy (PCV+) at 11 months of age displayed elastic lamina (EL) degradation, retinal pigment epithelium (RPE) atrophy, and photoreceptor degeneration. (A) Degradation of the EL of Bruch's membrane in Tg(BEST1-HTRA1)#Ybf/0 PCV+ mice. EL gaps are bracketed (Lower). (Scale bar, 1 um.) (B) Ultrastructure of the choroid, Bruch's membrane, and RPE of mutant PVC+ mice. Red brackets show RPE regions devoid of basal infoldings (Middle Left). Processes of an endothelial cell have inserted into EL gaps of mutant mice (Middle Right, red arrowheads). Red and yellow arrows (or arrowheads) show basal linear deposits and RPE vacuolization, respectively (Bottom). There were 45 RPE vacuoles in mutant vs. 4 in wild-type (WT). Twenty-six RPE regions from three WT and 24 RPE regions from three mutant PCV+ mice (one eye per mouse) were compared (1.88 +/- 0.28 per RPE region in PCV+ vs. 0.15 +/- 0.07 in WT, P < 0.001). (Scale bars, 1 um.) (C) Photoreceptor degeneration in mutant PCV+ mice. Red arrows and arrowheads indicate vacuolization between and within the innter segment of PCV+ mice, respectively. In both WT and mutant PCV+ mice, RPE is horizontal on the top (RPE was not included in the figure because of space limitation). (Scale bars, 2 um.)

Copyright

This image is from Jones A, Proc Natl Acad Sci U S A 2011 Aug 30;108(35):14578-83. Copyright 2011 National Academy of Sciences, U.S.A. J:175227

Associated Alleles

Symbol	Name
Tg(BEST1-HTRA1)#Ybf	transgene insertion, Yingbin Fu

Associated Genotypes

Allelic Composition	Genetic Background
Tg(BEST1-HTRA1)#Ybf/0	involves: C57BL/6J