Transgenic models for retinal degenerations
Strain | Gene | Mutation type | Cell layer | Degeneration time-course | Ref |
Mouse | |||||
B6D2F1 | rhodopsin | substitution (Pro23His) | ONL, RPE | starts at p20 | 34, 37 |
C57L/6J | rhodopsin | substitution (Pro347Ser) | ONL | p21–1 year | 20, 35 |
C57BL/6 × SJL | rhodopsin | substitution (Val20Gly, Pro23His, Pro27Leu) | ONL | p20–sp250 | 36, 40 |
C57L/6J | rhodopsin | nonsense (Gln344ter = Q344ter) | ONL | starts at p14 | 41 |
FVBN × C57BL/6 | peripherin | substitution (Pro216Leu) | ONL | 1 month–7 months | 42 |
C57BL/6J | IRBP | knockout (IRBP−/− ) | ONL | starts at p11 | 43 |
C57BL/6 × MF1 | γPDE | knockout (Pdeg−/− ) | ONL | p 0–8 weeks | 44 |
C57BL/6 | Rpe65 | knockout (Rpe65−/− ) | RPE, ONL | starts at 7 weeks | 52 |
C57BL/6 | — | insertion of diphtheria toxin A gene | ONL | p7–3 months | 54 |
C57BL/6 × 129Sv | cyclic nucleotidegated channel (CNG3) | knockout (CNG3−/− ) | ONL (rods) | 2 months–8 months | 55 |
C57BL/6 | ABCR | knockout (RmP−/− ) | ONL | — | 56 |
C57BL6 | rhodopsin | knockout (Rho−/− ) | ONL | p24–3 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, GCL | reduced developmental cell death at p 7 | 71 |
C3H/Bax−/− | Bax/β PDE | Bax−/− ,rd/rd | ONL | p 8–p 21 | 71 |
C3H × C57BL/6 | Pax2 | insertion (Pax2 1Neu) | OPL, INL, IPL, GCL | notable thinning in adult heterozygous mice | 118 |
129SvJ | GC-E | knockout (GC-E−/− ) | ONL | Starts at 3 weeks | 119 |
mouse (FVB/N × C57BL/6) | SV40 large tumour antigen | overexpression | ONL, OPL | p 5–p 100 | 120 |
Others | |||||
rat | rhodopsin | substitution (S344ter) | ONL | p 8–p20 | 30, 31 |
rat | rhodopsin | substitution (P23H) | ONL | p 15–1 year | 30, 32 |
pig | rhodopsin | substitution (Pro347Leu) | ONL | p 14–20 months | 33, 38, 39 |