Table 1.
Summary of inherited retinal degenerations (IRD) models and dysregulated components.
| Animal Model | Mutant Gene | Dysregulated Components | Related Retinal Degeneration | ER Stress Activation | Reference |
|---|---|---|---|---|---|
| Drosophila | RhoP23H | rhodopsin | ADRP | + | [33] |
| Xenopus laevis | RhoP23H | rhodopsin | RP | + | [54] |
| Rats | RhoP23H | rhodopsin | RP | + | [50] |
| Mice | RhoT17M | rhodopsin | ADRP | + | [28] |
| Transfected cell | ELOVL4 | an enzyme involved in the generation of long-chain fatty acids | Stargardt macular dystrophy | + | [39] |
| Rd1 mouse | PDE6-β | a catalytic subunit of a phosphodiesterase, regulating cGMP levels in photoreceptors | RP | + | [49] |
| ARPE-19 cells | R345W | N-Linked glycosylation | Malattia Leventinese and Doyne honeycomb retinal dystrophy | + | [55] |
ADRP, autosomal dominant retinitis pigmentosa; RP, retinitis pigmentosa; ER, endoplasmic reticulum.