![Figure 2. Ablation of Sam68 expression partially rescues viability, body weight, and motor function of SMAΔ7 mice. (A) Schematic diagram of transgenic mice crossing (top) and PCR analysis of genomic DNA (bottom) from tails of SMN2Δ7;SMN2;Smn+/+;Sam68+/+ (first lane), SMN2Δ7;SMN2;Smn−/−;Sam68+/+ (second lane), and SMN2Δ7;SMN2;Smn−/−;Sam68−/− (third lane) mice. Bands showing the transgenic and endogenous DNA bands amplified are indicated; the asterisk indicates an undefined band present in SMAΔ7 transgenic mice. (B) qPCR of SMN2Δ7 to quantify the dosage of transgenic SMN2Δ7 in hemizygous and homozygous transgenic animals. The data shown are from a single representative experiment, and values were normalized with Apolipoprotein B mRNA. (C) Micrograph showing representative non-SMA and SMAΔ7 (SMA) mice that are either wild type (Sam68+/+) or knockout (Sam68−/−) for Sam68. (D) Kaplan-Meier survival curves of SMAΔ7/Sam68+/+ (n = 50) and SMAΔ7/Sam68−/− (n = 22). Statistical analysis was performed by the log-rank test (P < 0.0001). (E) Weight curves of non-SMA/Sam68+/+, non-SMA/Sam68−/−, SMAΔ7/Sam68+/+, and SMAΔ7/Sam68−/− mice. Plot shows mean ± SD of at least 15 mice for each group for each day analyzed. The p-values were determined by a two-way ANOVA test followed by Bonferroni’s multiple comparison posttest. # represents the p-value of the non-SMA/Sam68+/+ versus non-SMA/Sam68−/− comparison; § represents the p-value of the non-SMA/Sam68−/− versus SMAΔ7/Sam68−/− comparison; and * represents the p-value of the SMAΔ7/Sam68+/+ versus SMAΔ7/Sam68−/− comparison. For all comparisons, P < 0.05, P < 0.01, P < 0.001, and P < 0.0001 are represented by increasing symbols (from one to four). (F) Time required for pups to stand up after being placed on their sides for SMAΔ7/Sam68+/+ mice compared with SMAΔ7/Sam68−/− mice at 8 and 10 dpp (nd [nondetected] indicates experimental animals that never stood up during the test). Statistical analysis was performed by two-way ANOVA test followed by Bonferroni’s multiple comparison posttest (*, P < 0.05; ***, P < 0.001).](https://cdn.rupress.org/rup/content_public/journal/jcb/211/1/10.1083_jcb.201502059/6/jcb_201502059_fig2.jpeg?Expires=1773549819&Signature=HFM9s9KRAxBqssuqMrk3g2H6ngwB97vUlZPBIfrfClwKev-hYsUfg2G60IxhS8-VSJn2gdso6c0iU6EzbxPyl3QU8qViboIn47-abD2Jsz4c8F3Mjpb6Pd6aM2zG9VQDJiYBWS0Z9DWbLRFHm55RcRit98YalBDrlijTMr8-wMTdixeU1D4Y4PHuvcl-zSyK9SS0fbwJzwhbqjDAFaLegUoy0etmC3JBcwFENjG-fM~PG9VERB~841BEoy27iwWZxLw3viINNmWPAr9Fq72uuy9CNPgl-U1-I4BLbf-rdW9KcYB-1AVQ-UWtf8d~86rShiTLfkOwXdBNgG3ec0dajQ__&Key-Pair-Id=APKAIE5G5CRDK6RD3PGA)
Ablation of Sam68 expression partially rescues viability, body weight, and motor function of SMAΔ7 mice. (A) Schematic diagram of transgenic mice crossing (top) and PCR analysis of genomic DNA (bottom) from tails of SMN2Δ7;SMN2;Smn+/+;Sam68+/+ (first lane), SMN2Δ7;SMN2;Smn−/−;Sam68+/+ (second lane), and SMN2Δ7;SMN2;Smn−/−;Sam68−/− (third lane) mice. Bands showing the transgenic and endogenous DNA bands amplified are indicated; the asterisk indicates an undefined band present in SMAΔ7 transgenic mice. (B) qPCR of SMN2Δ7 to quantify the dosage of transgenic SMN2Δ7 in hemizygous and homozygous transgenic animals. The data shown are from a single representative experiment, and values were normalized with Apolipoprotein B mRNA. (C) Micrograph showing representative non-SMA and SMAΔ7 (SMA) mice that are either wild type (Sam68+/+) or knockout (Sam68−/−) for Sam68. (D) Kaplan-Meier survival curves of SMAΔ7/Sam68+/+ (n = 50) and SMAΔ7/Sam68−/− (n = 22). Statistical analysis was performed by the log-rank test (P < 0.0001). (E) Weight curves of non-SMA/Sam68+/+, non-SMA/Sam68−/−, SMAΔ7/Sam68+/+, and SMAΔ7/Sam68−/− mice. Plot shows mean ± SD of at least 15 mice for each group for each day analyzed. The p-values were determined by a two-way ANOVA test followed by Bonferroni’s multiple comparison posttest. # represents the p-value of the non-SMA/Sam68+/+ versus non-SMA/Sam68−/− comparison; § represents the p-value of the non-SMA/Sam68−/− versus SMAΔ7/Sam68−/− comparison; and * represents the p-value of the SMAΔ7/Sam68+/+ versus SMAΔ7/Sam68−/− comparison. For all comparisons, P < 0.05, P < 0.01, P < 0.001, and P < 0.0001 are represented by increasing symbols (from one to four). (F) Time required for pups to stand up after being placed on their sides for SMAΔ7/Sam68+/+ mice compared with SMAΔ7/Sam68−/− mice at 8 and 10 dpp (nd [nondetected] indicates experimental animals that never stood up during the test). Statistical analysis was performed by two-way ANOVA test followed by Bonferroni’s multiple comparison posttest (*, P < 0.05; ***, P < 0.001).
