Table 2

Transgenic models for retinal degenerations

Strain Gene Mutation type Cell layer Degeneration time-course Ref
Mouse
 B6D2F1 rhodopsin substitution (Pro23His)ONL, RPEstarts at p20 34, 37
 C57L/6J rhodopsin substitution (Pro347Ser)ONLp21–1 year 20, 35
 C57BL/6 × SJL rhodopsin substitution (Val20Gly,  Pro23His, Pro27Leu)ONLp20–sp250 36, 40
 C57L/6J rhodopsin nonsense (Gln344ter =  Q344ter)ONLstarts at p14 41
 FVBN × C57BL/6 peripherin substitution (Pro216Leu)ONL1 month–7 months 42
 C57BL/6J IRBP knockout (IRBP−/− )ONLstarts at p11 43
 C57BL/6 × MF1γPDE knockout (Pdeg−/− )ONLp 0–8 weeks 44
 C57BL/6 Rpe65 knockout (Rpe65−/− )RPE, ONLstarts at 7 weeks 52
 C57BL/6insertion of diphtheria toxin A  geneONLp7–3 months 54
 C57BL/6 × 129Svcyclic nucleotidegated  channel (CNG3)knockout (CNG3−/− )ONL (rods)2 months–8 months 55
 C57BL/6 ABCR knockout (RmP−/− )ONL 56
 C57BL6 rhodopsin knockout (Rho−/− )ONLp24–3 months 57
 C57BL/6 rhodopsin kinase(RK)knockout (RK−/− )ONL (rods)depends on illumination state 59
 C57BL/6 × 129Sv arrestin knockout (arrestin−/− )ONL (only rods)3 months–12 months 60
 Bax−/− Bax knockout (Bax−/− )INL, GCLreduced developmental cell death at p 7 71
 C3H/Bax−/− Bax/β PDE Bax−/− ,rd/rd ONLp 8–p 21 71
 C3H × C57BL/6 Pax2 insertion (Pax2 1Neu)OPL, INL, IPL, GCLnotable thinning in adult heterozygous mice 118
 129SvJ GC-E knockout (GC-E−/− )ONLStarts at 3 weeks 119
 mouse (FVB/N ×  C57BL/6) SV40 large tumour  antigenoverexpressionONL, OPLp 5–p 100 120
Others
 rat rhodopsin substitution (S344ter)ONLp 8–p20 30, 31
 rat rhodopsin substitution (P23H)ONLp 15–1 year 30, 32
 pig rhodopsin substitution (Pro347Leu)ONLp 14–20 months33, 38, 39