Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy
J. Clin. Invest. Amy M. Avila, et al. 117:659 doi:10.1172/JCI29562 [
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Figure 6TSA increases survival, attenuates weight loss, and enhances motor behavior of SMA mice. SMA mice and their WT and heterozygous littermates were treated with daily intraperitoneal injections of TSA (10 mg/kg) or vehicle on days P5–P20. (
A) Four mice from the same litter at P13, showing the gross appearance of a TSA-treated SMA mouse, a vehicle-treated SMA mouse, a heterozygous (Het) mouse, and a WT mouse. (
B) Kaplan-Meier survival curves of mice treated with TSA (
n = 23) or vehicle (
n = 22).
P < 0.0003, log-rank test. (
C) Weights of SMA mice treated with TSA (
n = 26) or vehicle (
n = 22), heterozygous mice treated with TSA (
n = 52) or vehicle (
n = 49), and WT mice treated with TSA (
n = 30) or vehicle (
n = 30). (
D) Righting time in SMA mice treated with TSA (
n = 20) or vehicle (
n = 15), heterozygous mice treated with TSA (
n = 29) or vehicle (
n = 37), and WT mice treated with TSA (
n = 12) or vehicle (
n = 19).