Safety of Human USH1C Transgene Expression Following Subretinal Injection in Wild-Type Pigs

Invest Ophthalmol Vis Sci. 2025 Jan 2;66(1):48. doi: 10.1167/iovs.66.1.48.

Abstract

Purpose: This study aimed to evaluate early-phase safety of subretinal application of AAVanc80.CAG.USH1Ca1 (OT_USH_101) in wild-type (WT) pigs, examining the effects of a vehicle control, low dose, and high dose.

Methods: Twelve WT pigs (24 eyes) were divided into three groups: four pigs each received bilateral subretinal injections of either vehicle, low dose (3.3 × 1010 vector genomes [vg] per eye), or high dose (1.0 × 1011 vg per eye). Total retinal thickness (TRT) was evaluated using optical coherence tomography and retinal function was assessed with full-field electroretinography (ff-ERG) at baseline and two months post-surgery. After necropsy, retinal changes were examined through histopathology, and human USH1C_a1/harmonin expression was assessed by quantitative PCR (qPCR) and Western blotting.

Results: OT_USH_101 led to high USH1C_a1 expression in WT pig retinas without significant TRT changes two months after subretinal injection. The qPCR revealed expression of the human USH1C_a1 transgene delivered by the adeno-associated virus vector. TRT changes were minimal across groups: vehicle (256 ± 21 to 243 ± 18 µm; P = 0.108), low dose (251 ± 32 to 258 ± 30 µm; P = 0.076), and high dose (242 ± 24 to 259 ± 28 µm; P = 0.590). The ff-ERG showed no significant changes in rod or cone responses. Histopathology indicated no severe retinal adverse effects in the vehicle and low dose groups.

Conclusions: Early-phase clinical imaging, electrophysiology, and histopathological assessments indicated that subretinal administration of OT_USH_101 was well tolerated in the low-dose treatment arm. OT_USH_101 treatment resulted in high expression of human USH1C_a1. Although histopathological changes were not severe, more frequent changes were observed in the high-dose group.

MeSH terms

  • Animals
  • Blotting, Western
  • Cell Cycle Proteins / genetics
  • Cytoskeletal Proteins / genetics
  • Dependovirus / genetics
  • Disease Models, Animal
  • Electroretinography*
  • Gene Expression Regulation
  • Genetic Therapy / methods
  • Genetic Vectors*
  • Humans
  • Injections, Intraocular*
  • Real-Time Polymerase Chain Reaction
  • Retina* / metabolism
  • Retina* / pathology
  • Swine
  • Tomography, Optical Coherence*
  • Transgenes*

Substances

  • Cell Cycle Proteins
  • Cytoskeletal Proteins