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Related Experiment Video

Updated: Feb 21, 2026

Application of Optical Coherence Tomography to a Mouse Model of Retinopathy

Application of Optical Coherence Tomography to a Mouse Model of Retinopathy

Published on: January 12, 2022

Retinal pathology in the PPCD1 mouse.

Anna L Shen^1,2, Susan M Moran¹, Edward A Glover¹

¹The McArdle Laboratory for Cancer Research, Department of Oncology, School of Medicine and Public Health, University of Wisconsin-Madison, Madison, Wisconsin, United States of America.

|October 6, 2017

Summary

Posterior polymorphous corneal dystrophy (PPCD1) mouse models exhibit elevated intraocular pressure (IOP) and significant retinal damage, including cell loss and detachment. These retinal defects are independent of the GpnmbR150X mutation.

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Area of Science:

Ophthalmology
Genetics
Mouse Models

Background:

Posterior polymorphous corneal dystrophy (PPCD) is a rare inherited eye disorder.
The PPCD1 mouse model on a DBA/2J background (D2.Ppcd1) develops anterior segment abnormalities.
Previous studies noted enlarged anterior chambers due to corneal endothelium issues.

Purpose of the Study:

To characterize the retinal phenotypes in D2.Ppcd1 mice.
To investigate the relationship between PPCD1, intraocular pressure (IOP), and retinal degeneration.
To determine if the GpnmbR150X mutation influences the development of these defects.

Main Methods:

Phenotypic characterization of D2.Ppcd1 mice.
Intraocular pressure (IOP) measurements at various ages.
Histopathological examination of retinal layers.

Main Results:

D2.Ppcd1 mice showed significantly increased IOP by three months of age.
Retinal ganglion cell layer loss was observed by five months.
Retinal detachment was evident as early as three weeks, with outer nuclear layer degeneration and RPE hyperplasia observed later.
Anterior segment and retinal defects occurred independently of the GpnmbR150X mutation.

Conclusions:

D2.Ppcd1 mice serve as a valuable model for studying PPCD-associated ocular complications.
The study highlights a link between corneal endothelial dysfunction and progressive retinal damage, including glaucoma.
The findings indicate that the genetic basis for PPCD1-induced retinal defects in this model is distinct from the GpnmbR150X mutation.