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. Author manuscript; available in PMC: 2017 May 12.
Published in final edited form as: Gene Ther. 2017 Feb 2;24(4):215–223. doi: 10.1038/gt.2017.4

Table 1.

Anatomical measures in hearts of CLN2-affected dogs that received ICV TPP1 Gene Therapy

Measurement Dog 7*
10 Mos
Dog 6*
10 Mos
Dog 4
18 Mos
Dog 1
18 Mos
Dog 2
21 mos
Reference Range
H/BW %1 0.78% 0.74% 1.13% ↑ 0.93 0.94 ↑ 0.58–0.94%
LV+S/RV2 2.86 3.18 2.36 ↓ 2.4↓ 2.42 ↓ 2.76–3.88
RV/BW, %3 0.18 0.16 0.27 ↑ 0.23 ↑ 0.24 ↑ 0.1–0.18
LV+S/BW, %4 0.54 0.49 0.65 ↑ 0.56 ↑ 0.59 ↑ 0.35–0.55
RV/TC, %5 23.8 ↑ 21.0 ↑ 24% ↑ 24.9↑ 25.7 ↑ 15.3–20.9%
LV+S/TC, %6 68.2 ↑ 66.8 ↑ 56 59.7 62.3 52.5–66.5%
1

Total heart weight/body weight

2

Left ventricle and septum weight/right ventricle weight

3

Right ventricle weight/body weight

4

Left ventricle and septum weight/body weight

5

Right ventricle weight/total cardiac weight

6

Left ventricle and septum weight/total cardiac weight

*

Dogs 6 and 7 were untreated affected dogs; dogs 1, 2 and 4 correspond to the dogs listed in Figures 13