You are currently viewing a new version of our website. To view the old version click .

4 Results Found

  • Article
  • Open Access
818 Views
13 Pages

11 July 2025

Background/Objective: The Rs1 exon-1-del rat (Rs1KO) XLRS model shows normal retinal development until postnatal day 12 (P12) when small cystic spaces start to form in the inner nuclear layer. These enlarge rapidly, peak at P15, and then collapse by...

  • Review
  • Open Access
9 Citations
7,091 Views
37 Pages

The Road towards Gene Therapy for X-Linked Juvenile Retinoschisis: A Systematic Review of Preclinical Gene Therapy in Cell-Based and Rodent Models of XLRS

  • Isa van der Veen,
  • Andrea Heredero Berzal,
  • Céline Koster,
  • Anneloor L. M. A. ten Asbroek,
  • Arthur A. Bergen and
  • Camiel J. F. Boon

19 January 2024

X-linked juvenile retinoschisis (XLRS) is an early-onset progressive inherited retinopathy affecting males. It is characterized by abnormalities in the macula, with formation of cystoid retinal cavities, frequently accompanied by splitting of the ret...

  • Article
  • Open Access
3 Citations
1,690 Views
20 Pages

Kir4.1 and Aqp4 Contribution to Schisis Cystic Water Accumulation and Clearance in the Rs1 Exon-1 Del XLRS Rat Model

  • Zeljka Smit-McBride,
  • Ning Sun,
  • Serafina Thomas,
  • In Hwan Cho,
  • Robin G. Stricklin and
  • Paul A. Sieving

9 December 2024

Background/Objective: The Rs1 exon-1-del rat (Rs1KO) XLRS model shows normal retinal development until postnatal day 12 (P12) when small cystic spaces start to form in the inner nuclear layer. These spaces enlarge rapidly, peak at P15, and then colla...

  • Article
  • Open Access
4 Citations
2,473 Views
21 Pages

Early Developmental Characteristics and Features of a Three-Dimensional Retinal Organoid Model of X-Linked Juvenile Retinoschisis

  • Jung Woo Han,
  • Hun Soo Chang,
  • Sung Chul Park,
  • Jin Young Yang,
  • Ye Ji Kim,
  • Jin Ha Kim,
  • Hyo Song Park,
  • Han Jeong,
  • Junwon Lee and
  • Chang Ki Yoon
  • + 4 authors

X-linked juvenile retinoschisis (XLRS) is a hereditary retinal degeneration affecting young males caused by mutations in the retinoschisin (RS1) gene. We generated human induced pluripotent stem cells (hiPSCs) from XLRS patients and established three...