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M. J. Fenberg, M. L. Katz, D. N. Sanders; Characterization of Retinal Degeneration in Mouse Models Affected With Neuronal Ceroid Lipofuscinosis Using Optical Coherence Tomography. Invest. Ophthalmol. Vis. Sci. 2008;49(13):2168.
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Mouse models are available for many inherited diseases that involve progressive retinal degeneration. Among these are infantile and juvenile neuronal ceroid lipofuscinoses (NCLs) that result from mutations in the CLN1 and CLN3 genes respectively. Therapeutic interventions to prevent retinal degeneration are being evaluated in these models. The purpose of our study is to determine whether optical coherence tomography (OCT) can be used to assess the efficacy of these interventions.
Characterization of retinal changes in CLN1 and CLN3 knockout mice of varying ages were performed using OCT and compared with age-matched unaffected controls. Affected mice that had received intraocular stem cell implants were also evaluated. Histological analyses were then performed to validate OCT measurements.
High quality OCT images of the mouse retina could be obtained using a standard clinical OCT instrument with minimal modification. Measurements of retinal thickness were reproducible, both within an eye and between different animals of the same genotype and age. OCT measurements correlated well with histological measurements. Thus, changes in retinal thickness could be followed over time in the same animals. In addition, images of cells implanted into the vitreous cavity could be obtained with OCT, allowing noninvasive monitoring of donor cell migration in the mouse eye.
Standard clinical OCT can be used to reliably assess retinal thickness and monitor donor cell migration in the mouse model. OCT instrumentation will allow the progression of retinal degeneration and the effects of therapeutic intervention to be followed over time.
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