Negative influence of PAX3-FKHR on Pax3 activity. Kaplan–Meier
survival plots of offspring resulting from HA21 ×
Splotch matings show that HA21
Tg+Sp± mice had a significantly reduced
lifespan (A). The survival of
Tg−Sp+/+ (row 2, n = 20),
Tg−Sp± (row 1, n = 20),
Tg+Sp+/+ (row 3, n = 23),
and Tg+Sp± (row 4, n = 9)
mice was monitored over the course of 120 days. PAX3 and PAX3-FKHR
compete for transcriptional activity (B). When
increasing amounts of PAX3 were added (50 ng, 100 ng, and 1 μg),
PAX3-FKHR transactivation of PRS-9 was blocked to the levels of PAX3
alone. The activity from PAX3-FKHR alone was arbitrarily set at
100%.