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Figure 1.

Experimental design for the study.

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Table 1.

The macronutrient information of each diet.

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Table 2.

The composition of the metabolic therapy used in this study.

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Table 3.

Criteria of the neurological score.

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Table 4.

The mean weight of organs and tissue samples collected from each group.

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Figure 2.

Effect of treatment on body weight in SOD1-G93A mice.

There was no significant difference in body weight of SOD1-G93A mice between the different diet groups either before treatment started (Baseline), at presymptomatic stage (before neurological score 1) and at endpoint of the study. All data are mean ± SEM.

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Figure 3.

Improved motor function was observed in KD and SD+DP groups.

A) KD group had significantly better result on accelerating rotarod during week 11 and 15 (p = 0.03, p = 0.018, respectively) when compared to the control group. (SD, n = 12; SD+DP, n = 11; KD, n = 9; KDDP, n = 9). B) Grip test showed better motor performance in KD group at week 15 (p = 0.015), while the motor function was significantly better at week 17 in SD+DP group (p = 0.039), compared to control. (SD, n = 13; SD+DP, n = 12; KD, n = 11; KDDP, n = 12). C) PaGE test showed better motor performance in KD group on week 15 and 16 (p = 0.027, p = 0.01), while the motor function was significantly better at week 12, 15, 16 and 17 in SD+DP group (p = 0.033, p = 0.023, p = 0.002, p = 0.019, respectively), when compared to SD group. All data are mean ± SEM. (SD, n = 9; SD+DP, n = 8; KD, n = 8; KDDP, n = 8). (*shows significant difference between SD and SD+DP groups; # shows significant difference between SD and KD+DP groups).

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Figure 4.

SD+DP delayed disease progression in SOD1-G93 mice.

The difference in neurological score was significant between the control group (SD) and KD group on day 105 and 106, and the SD+DP group between day 118 and day 128 (except no data on day 119, 123, 124) implicating delayed disease progression in SD+DP group (p <0.05). All data are mean ± SEM.

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Figure 5.

SD+DP increased survival time in SOD1-G93 mice.

Kaplan-Meier survival plot of study groups. Animals receiving SD+DP showed significantly longer survival compared to control animals (p = 0.001).

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Table 5.

The mean age at endpoint, increase in lifespan and the number of animals survived past 125 days are shown for each group.

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