Raw data and summary of endpoint criteria for 162 B6SJL-TgN-(SOD1-G93A)1Gur autosomal hemizygous female (F) and male (M) mice.

<p>CS 4, clinical score of 4 = functional paralysis of both hindlimbs; 20%CS2, weight loss ≥20% <i>vs.</i> body weight immediately prior to a clinical score of 2; 20%Peak, weight loss ≥20% <i>vs.</i> peak body weight; BC<2, body condition score <2; CS 5, clinical score of 5 = CS 4 plus a righting reflex >20 s; CS4+, clinical score of 4+ = CS 4 in addition to the earliest of 20%CS2, 20%Peak, BC<2 or a righting reflex of >20 s.</p><p>*Significantly different from each other (P<0.001). Data for Mean Age (d) are presented as means ± SD.</p

Raw data and summary of 162 B6SJL-TgN-(SOD1-G93A)1Gur autosomal hemizygous female (F) and male (M) mice meeting the additional endpoint criteria prior to reaching CS 4.

<p>20%CS2, weight loss ≥20% <i>vs.</i> body weight immediately prior to a clinical score of 2; 20%Peak, weight loss ≥20% <i>vs.</i> peak body weight; BC<2, body condition score <2.</p><p>*Earliest age (d) of 20%CS2, 20%Peak and BC<2 was used to calculate mean age. Data for Mean Age (d) are presented as means ± SD.</p

Correlation between CS 4 and CS 4+ and the Bland-Altman plot for CS 4 and CS 4+.

<p>(A) Correlation between CS 4 (clinical score of 4 = functional paralysis of both hindlimbs) and CS 4+ [CS 4 plus the earliest of a) weight loss ≥20% <i>vs.</i> body weight immediately prior to a clinical score of 2, b) weight loss ≥20% <i>vs.</i> peak body weight, c) body condition score <2, or d) a righting reflex >20 s (CS 5)]. There was a strong positive relationship between CS 4 and CS 4+ (r = 0.96, slope = 0.92, P<0.001). CS 4+ (d) = (12.79±2.56)+[(0.92±0.02)×(CS 4 in d)], mean ± SEM. Dashed line indicates line of identity. (B) A Bland-Altman plot comparing CS 4 to CS 4+. Mean bias ± SD = 2.2±2.3%, lower limit = −2.4%, upper limit = 6.7%.</p

Correlation between CS 5 and CS 4+ and the Bland-Altman plot for CS 5 and CS 4+.

<p>(A) Correlation between CS 5 (clinical score of 5 = CS 4 and righting reflex >20 s) and CS 4+ [CS 4 plus the earliest of a) weight loss ≥20% <i>vs.</i> body weight immediately prior to a clinical score of 2, b) weight loss ≥20% loss <i>vs.</i> peak body weight, c) body condition score <2, or d) a righting reflex >20 s (CS 5)]. There was a strong positive relationship between CS 5 and CS 4+ (r = 0.98, slope = 0.98, P<0.001). CS5 (d) = (3.93±2.15)+[(0.98±0.02)×(CS 4+ in d)], mean ± SEM. Dashed line indicates line of identity. (B) A Bland-Altman plot comparing CS 5 to CS 4+. Mean bias ± SD = 0.8±1.7%, lower limit = −2.5%, upper limit = 4.1%.</p

Probability of survival for CS 4, CS 4+ and CS 5.

<p>Probability of survival for the 3 different endpoints (CS 4, black line; CS 4+, blue line; CS 5, red line). For all logrank tests, CS 4 was used as the reference when comparing CS 4 vs. CS 4+ and CS 4 vs. CS 5, whereas CS 4+ was used as the reference when comparing CS 4+ vs. CS 5. The rate of reaching endpoint is significantly different (P = 0.021) between CS 4 (clinical score of 4 = functional paralysis of both hindlimbs), CS 4+ [CS 4 plus the earliest of a) weight loss ≥20% <i>vs.</i> body weight immediately prior to a clinical score of 2, b) weight loss ≥20% <i>vs.</i> peak body weight, c) body condition score <2, or d) a righting reflex >20 s (CS 5)], and CS 5 (clinical score of 5 = CS 4 and righting reflex >20 s). Mice reached CS 4 at a rate of 34% faster vs. CS 5 (HR = 1.34; 95% CI 1.10, 1.74; P = 0.006) and 24% faster vs. CS 4+ (HR = 1.24; 95% CI 1.00, 1.59; P = 0.046). Mice reached CS 4+ at a non-significant rate of 9% faster vs. CS 5 (HR = 1.09; 95% CI 0.88, 1.38; P = 0.410).</p

Correlation between CS 4 and CS 5 and the Bland-Altman plot for CS 4 vs. CS 5.

<p>(A) Correlation between CS 4 (clinical score of 4 = functional paralysis of both hindlimbs) and CS 5 (clinical score of 5 = CS 4 plus a righting reflex >20 s). There was a strong positive relationship between CS 4 and CS 5 (r = 0.95, slope = 0.91, P<0.001). CS 5 (d) = (14.80±2.83)+[(0.91±0.02)×(CS 4 in d)], mean ± SEM. Dashed line indicates line of identity. (B) A Bland-Altman plot comparing CS 4 to CS 5. Mean bias ± SD = 3.0±2.5%, lower limit = −2.0%, upper limit = 7.9%.</p

No sex differences in PPARα or PPARγ protein content.

<p>Protein content of PPARα (A) and PPARγ (B) in skeletal muscle of men and women, adjusted to actin. Representative western blots; lanes 1–4 are men, lanes 5–8 women. N = 12 men and 11 women.</p

Subject characteristics.

<p>Data are means ±SE. a; significantly lower in women P≤0.05, b; significantly higher in women P≤0.05.</p

SCHAD protein content is equal in women and men.

<p>SCHAD mRNA content between men and women shown by Real time RT-PCR, adjusted to β2-M mRNA (A). Protein content of SCHAD in skeletal muscle of men and women, adjusted to CS (B,C). Representative western blot (B) Lanes 1–4 are men, lanes 5–8 women. N = 12 men and 11 women.</p

TFPα protein content is higher in women than men.

<p>Differences in TFPα mRNA content between men and women shown by Real time RT-PCR, adjusted to β2-M mRNA (A). Protein content of TFPα in skeletal muscle of men and women, adjusted to CS (B,C). Representative western blot (B) Lanes 1–5 are men, lanes 6–10 are women. N = 12 men and 11 women. *P≤0.05.</p