Retinal and choroidal neovascularization in a transgenic mouse model of sickle cell disease

Gerard A. Lutty, D. Scott McLeod, Agathe Pachnis, Frank Costantini, Mary E. Fabry, Ronald L. Nagel

Research output: Contribution to journalArticlepeer-review

28 Scopus citations


A complication of sickle cell disease is proliferative retinopathy. We investigated the eyes from a transgenic mouse model of sickle cell disease (α(H)β(S)[β(MDD)] type) to determine if pathological changes occurred in their retinas and choroids. One retina from each animal was processed by flat-embedding adenosine diphosphatase-reacted retinas in glycol methacrylate. The fellow eye from each animal was embedded whole in glycol methacrylate for histopathological analysis of all ocular structures. Retinal vascular occlusions resulted in nonperfused areas of retina and arterio- venous anastomoses. Intra- and extraretinal neovascularization was observed adjacent to nonperfused areas. Retinal pigmented lesions were formed by the migration of retinal pigment epithelial cells into sensory retina, often ensheathing choroidal neovascularization. The incidence of this bilateral chorioretinopathy was 30% in animals older than 15 months of age. The ocular histopathological changes we observed in the mouse model mimicked many aspects of human proliferative sickle cell retinopathy. Furthermore, this is the first genetically derived animal model for chorio-retinal neovascularization.

Original languageEnglish (US)
Pages (from-to)490-497
Number of pages8
JournalAmerican Journal of Pathology
Issue number2
StatePublished - Aug 1994
Externally publishedYes

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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