Retinal Glial and Choroidal Vascular Pathology in Donors Clinically Diagnosed With Stargardt Disease

Investigative Ophthalmology & Visual Science
Malia M EdwardsGerard A Lutty

Abstract

The present study investigated retinal glia and choroidal vessels in flatmounts and sections from individuals with clinically diagnosed Stargardt disease (STGD). Eyes from three donors clinically diagnosed with STGD were obtained through the Foundation Fighting Blindness (FFB). Genetic testing was performed to determine the disease-causing mutations. Eyes were enucleated and fixed in 4% paraformaldehyde and 0.5% glutaraldehyde. After imaging, retinas were dissected and immunostained for glial fibrillary acidic protein, vimentin, and peanut agglutin. Following RPE removal, the choroid was immunostained with Ulex europaeus agglutinin lectin. For each choroid, the area of affected vasculature, percent vascular area, and choriocapillaris luminal diameters were measured. The retina from one donor was hemisected and cryopreserved or embedded in JB-4 for cross-section analysis. Genetic testing confirmed the STGD diagnosis in donor 1, whereas a mutation in peripherin 2 was identified in donor 3. Genetic testing was not successful on donor 2. Therefore, only donor 1 can definitively be classified as having STGD. All donors had areas of RPE atrophy within the macular region, which correlated with underlying choriocapillaris loss. In addi...Continue Reading

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Jul 30, 2015·Experimental Eye Research·Malia M EdwardsGerard A Lutty
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Jul 28, 2016·Ophthalmology·Marco PellegriniGiovanni Staurenghi
Nov 2, 2016·Investigative Ophthalmology & Visual Science·D Scott McLeodGerard A Lutty
Feb 23, 2017·Retina·Imoro Z BraimahJay Chhablani
Mar 2, 2017·Investigative Ophthalmology & Visual Science·Malia M EdwardsGerard A Lutty
Jul 25, 2017·Investigative Ophthalmology & Visual Science·Preena TannaMichel Michaelides
Oct 4, 2017·Investigative Ophthalmology & Visual Science·Enrico BorrelliSriniVas R Sadda
Dec 14, 2019·Translational Vision Science & Technology·Alessandro ArrigoMaurizio Battaglia Parodi

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Citations

Jul 23, 2021·Progress in Retinal and Eye Research·Raphael LejoyeuxJay Chhablani
Oct 30, 2021·Graefe's Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Für Klinische Und Experimentelle Ophthalmologie·Vijay K JidigamSujata Rao

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