Figure 7.

Western blotting and behavior analysis of HD KI‐140Q mice intravenously injected with SM3. a) Western blotting analysis of HD KI‐140Q and WT mice injected with GFP or SM3 using antibodies to LAMP1, P62, Beclin 1, and LC3. Vinculin served as a loading control. b) Quantification of the ratios of LAMP1, P62, Beclin 1, and LC3II to vinculin on the western blots. The data were obtained from four independent experiments (n = 4). Data were analyzed by one‐way ANOVA with Dunnett's multiple comparisons test and presented as mean ± SEM. LAMP1: **P =  0.0040; *P =  0.0359. P62: **P =  0.0038. Beclin 1: **P =  0.0011; *P =  0.0302. LC3: **P =  0.0020; *P =  0.0481. c) Western blotting analysis of the HD KI‐140Q mouse striatum to detect mHTT in the purified lysosome fraction. Full‐length mHTT was detected by 1C2 antibody and indicated by an arrow. The blots were also probed with LAMP1 and vinculin antibodies. d) Quantification of the ratios of mHTT to vinculin (total protein) or LAMP1 (lysosome) on the western blots. The data were obtained from four independent experiments (n = 4). Data were analyzed by two‐tailed Student's t test and presented as mean ± SEM. *P =  0.0174; ***P =  0.0006. e) ELISA assay of lysosomal enzyme activity. n = 4 mice per group. Data were analyzed by one‐way ANOVA with Dunnett's multiple comparisons test and presented as mean ± SEM. *P = 0.0424 (WT‐GFP vs KI‐140Q‐GFP); *P = 0.0358 (KI‐140Q‐GFP vs KI‐140Q‐SM3). f–i) Evaluation of motor functions of HD KI‐140Q mice one month after intravenous injection of GFP (KI‐140Q‐GFP) or SM3 (KI‐140Q‐SM3) using f) forelimbs grip, g) fore and hind limbs grip, h) balance beam, and i) rotarod tests. Data were analyzed by two‐tailed Student's t test and presented as mean ± SEM. **P = 0.005 (fore and hind limbs grip); *P = 0.0279 (balance beam); *P = 0.0105 (rotarod). n = 12 mice per group.