Table 2.
Current Engineered Mouse Models with CAV3 Mutations
| Reference | CAV3 mutation | Model intended for: | Actual phenotype hetero- or hemizygous |
Homozygous |
|---|---|---|---|---|
|
Hagiwara, el al. 2000; Woodman, el al. 2002 |
Null | AR-caveolinopathy | Normal | Skeletal myopathy after 8 wks; progressive cardiomy- opathy beginning at 8 wks |
| Galbiati, et al. 2000; Aravamuden, et al. 2003 | CAV3 over-expression using CAG promoter |
Model of CAV3 over- expression seen in DMD and mdx mice |
Skeletal myopathy by 3–4 wks; cardiomyopathy by 6–10 wks; death by 10–12 months |
NA |
| Tsutsumi, et al. 2008; Horikawa, et al. 2011 | CAV3 over-expression using a- MHC promoter to drive cardio- myocyte expression |
Examining effects of CAV3 on natriuretic pep- tide signaling |
Normal to beneficial | NA |
| Sunada, el al. 2001; Ohsawa, et al. 2004 | P104L transgenic using MCK promoter |
LGMD1C | Skeletal myopathy by 6 wks; hindlimb paralysis by 12 wks; hypertrophic cardiomyopathy by 6 wks |
NA |