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. 2021 Nov 8;13(12):e14397. doi: 10.15252/emmm.202114397

Figure 2. Uqcrh −/− mice displayed several essential characteristics of human CIII disorders.

Figure 2

  • A
    Representative picture of a WT and age‐ and sex‐matched Uqcrh −/− mouse at 4 weeks of age. Scale expressed in centimetres.
  • B
    Body weight of Uqcrh −/− mice and wild‐type littermates at 1–2 weeks (n = 10 f, 5 m Uqcrh −/− and 8 f, 8 m WT); during the post‐weaning period from 5 to 6 weeks of age (n = 3 m, 7 f Uqcrh −/− and 3 f, 3 m WT); 6–7 weeks of age (n = 28 f, 23 m Uqcrh −/− and 22 f, 23 m WT); 7–8 weeks of age (n = 33 f, 30 m Uqcrh −/− and 45 f, 40 m WT); 8–9 weeks of age (n = 21 f, 25 m Uqcrh −/− and 35 f, 37 m WT); 9–10 weeks of age (n = 45 f, 45 m Uqcrh −/− and 61 f, 66 m WT); 10–11 weeks of age (n = 14 f, 13 m Uqcrh −/− and 21 f, 25 m WT). Data are shown as mean ± SD. ****P < 0.0001, 2‐way ANOVA, from 7.5 weeks of age for Uqcrh −/− males and females versus sex‐matched controls.
  • C, D
    Clinical chemistry plasma concentrations of parameters related to acid‐base balance (lactate and ammonia) and metabolic state (glucose) n = 15 Uqcrh −/− and 16 WT pups 1–2‐week‐old (C); n = 15 f, 15 m Uqcrh −/− and 15 f,15 m WT mice aged 8–9 weeks (D); sex not differentiated. Data are shown as scatter dot plots with a line at the median. *P < 0.05, **P < 0.01, ****P < 0.0001, Wilcoxon rank‐sum test.
  • E, F
    Measures related to behaviour and neurological function, data generated by the open‐field test (8 weeks old, E) and SHIRPA (9 weeks old, F), n = 7f, 8m Uqcrh −/− and 8f, 9m WT animals. Prepulse inhibition of the acoustic startle as a parameter associated with sensorimotor gating (10 weeks, E right panel), n = 7 f/5 m Uqcrh −/− and 8 f/ 9 m WT mice. Sex is not differentiated. Data are shown as scatter dot plots with a line representing the median. ***P < 0.001, ****P < 0.0001, Wilcoxon rank‐sum test.
  • G
    Parameters related to heart function generated by echocardiography (ECHO) and electrocardiography (ECG), n = 15 f, 15 m Uqcrh −/− and 15 f, 15 m WT mice, 6 weeks old.

Data information: Data are shown as single data points and median, or categorical data, males and females pooled, since genotype‐related effects were comparable for both sexes from different cohorts. *P < 0.05, ***P < 0.001, Wilcoxon rank‐sum test.