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. Author manuscript; available in PMC: 2019 May 1.
Published in final edited form as: Mol Genet Metab. 2018 Mar 27;124(1):71–81. doi: 10.1016/j.ymgme.2018.03.011

Fig. 4.

Fig. 4

Impaired mitochondrial respiration functions in the fibroblasts of the proband. (A) Profile of the oxygen consumption rate (OCR) adapted from the Agilent Technologies brochure of the Mitochondrial Stress Test. (B) Compared OCR responses among the proband, his mother, and three healthy control subjects. (C) Quantitative data between the proband and the age-matched control (10-month-old infant) of basal respiration, ATP-linked respiration, maximal respiration, and spare respiratory capacity. Data are represented as means ± S.D., n = 3 of three independent experiments. * and ** indicate statistically significant differences with a p value of ≤0.005 and ≤0.0012, respectively, between the proband and healthy infant. (D) Quantitative data between the proband and his mother of basal respiration, ATP-linked respiration, maximal respiration, and spare respiratory capacity. Data are represented as means ± S.D., n = 3 of independent experiments. * and ** indicate statistically significant differences with a p value of 0.0003 and ≤0.0019, respectively, between the proband and his mother.