Table 1 FSHD-like mouse severity models
Genotype | TMX | Transgene recombination | DUX4-fl expression | Phenotype |
|---|---|---|---|---|
ACTA1-MCM/+ | 5 mg/kg × 1 IP | NA | No | Control moderate |
ACTA1-MCM/+ | 10 mg/kg × 2 IP | NA | No | Control Severe |
ACTA1-MCM;FLExD/+ | None | 2-10% | Yes | Mild |
ACTA1-MCM;FLExD/+ | 5 mg/kg × 1 IP | 7–28% | Yes | Moderate |
ACTA1-MCM;FLExD/+ | 10 mg/kg × 2 IP | 38–55% | Yes | Severe |