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

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

Abstract

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 Cln3Δex7/8 mouse retina at as young as 8 month old. In this data article, we performed differential interference contrast and confocal imaging analyses in all retinal layers to localize and characterize these autofluorescent lesions, including their spectral characteristics and morphology. We further studied colocalization of these autofluorescent lesions with the JNCL marker mitochondrial ATP synthase F0 sub-complex subunit C and various established retinal cell type markers.

Original languageEnglish
Article number106076
JournalData in Brief
Volume32
DOIs
StatePublished - Oct 2020

Bibliographical note

Funding Information:
This work was supported by National Institutes of Health Center of Biomedical Research Excellence (COBRE) award P20GM121327 (pilot grant to Q.J.W.), and National Institutes of Health EY028206 and BrightFocus Foundation (to M.E.K.). Q.J.W. also thank Drs. Susan L. Cotman (Massachusetts General Hospital) and Wai T. Wong (National Eye Institute, NIH) for suggestions of subunit C and IBA1 antibodies, respectively .

Publisher Copyright:
© 2020

Keywords

  • CLN3
  • Juvenile Neuronal Ceroid Lipofuscinosis
  • autofluorescent lesions
  • mitochondrial ATP synthase F0 sub-complex subunit C
  • retinopathy
  • vision loss

ASJC Scopus subject areas

  • General

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