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Prominin-1 null Xenopus laevis develop subretinal drusenoid-like deposits, cone-rod dystrophy, and RPE atrophy.

Brittany J Carr1, Dominic Skitsko2, Jun Song3

  • 1The University of Alberta, Faculty of Medicine and Dentistry, Department of Ophthalmology and Visual Sciences.

Biorxiv : the Preprint Server for Biology
|June 19, 2024
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Summary

Mutations in the PROMININ-1 gene cause vision loss. This study models the condition in frogs, revealing retinal pigment epithelium damage precedes vision degeneration, marked by drusen-like deposits.

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Area of Science:

  • Genetics and Ophthalmology
  • Xenopus laevis disease modeling

Background:

  • Mutations in PROMININ-1 (PROM1) are linked to inherited, non-syndromic vision loss.
  • The precise mechanism of PROM1-associated retinal degeneration remains unclear.

Purpose of the Study:

  • To create and characterize a Xenopus laevis model of PROM1-null mediated retinal degeneration.
  • To investigate the age-dependent progression and underlying pathology of this vision loss model.

Main Methods:

  • CRISPR/Cas9 gene editing to induce prom1-null mutations in Xenopus laevis.
  • Longitudinal tracking of retinal degeneration from 6 weeks to 3 years.
  • Histological analysis, optical coherence tomography (OCT), and color fundus photography.

Main Results:

  • PROM1-null frogs exhibit age-dependent retinal degeneration.
  • Subretinal drusenoid deposit (SDD)-like structures accumulate with age, preceding photoreceptor degeneration.
  • Evidence suggests retinal pigment epithelium (RPE) dysfunction and thinning contribute to deposit formation.

Conclusions:

  • PROM1-null mediated blindness involves RPE death and dysfunction preceding photoreceptor degeneration.
  • The Xenopus model recapitulates key features of human retinal diseases with SDD-like deposits.
  • This model provides new insights into the pathogenesis of PROM1-associated inherited retinal diseases.