Ophthalmology Commons^™

Imaging Data On Characterization Of Retinal Autofluorescent Lesions In A Mouse Model Of Juvenile Neuronal Ceroid Lipofuscinosis (Cln3 Disease), Qing Jun Wang, Kyung Sik Jung, Kabhilan Mohan, Mark E. Kleinman

Ophthalmology and Visual Science Faculty Publications

Juvenile neuronal ceroid lipofuscinosis (JNCL, aka. juvenile Batten disease or CLN3 disease), a lethal pediatric neurodegenerative disease without cure, often presents with vision impairment and characteristic ophthalmoscopic features including focal areas of hyper-autofluorescence. In the associated research article “Loss of CLN3, the gene mutated in juvenile neuronal ceroid lipofuscinosis, leads to metabolic impairment and autophagy induction in retinal pigment epithelium” (Zhong et al., 2020) [1], we reported ophthalmoscopic observations of focal autofluorescent lesions or puncta in the Cln3^Δex7/8 mouse retina at as young as 8 month old. In this data article, we performed differential interference contrast and …