3.1.1. Mito Stress Test

Without any additional FAs, almost all important parameters of mitochondrial function, such as basal respiration (BR), maximal respiration (MR) and spare respiratory capacity (SRC) were significantly lower in patients compared to controls. Only the ATP linked respiration rate (ATP-R) was not significantly different in patients and controls (Figure 1, Table 2).

≤ ≤ ≤ **Figure 1.** Evaluation of mitochondrial function using a Seahorse XF96 Cell Analyzer in myoblasts from patients (*n* = 5) and controls (*n* = 5). Key parameters of mitochondrial function were analyzed as described in methods. (**A**) basal respiration (BR), (**B**) maximal respiration (MR), (**C**) ATP linked respiration (ATP-R), and (**D**) spare respiratory capacity (SRC). In some experiments, FAs oleate or palmitate were added as excessive nutrients. The significant differences between patients and controls are presented in Table 2. The significance between values without FAs or after treatment with them is only shown if there are significant differences: \* = *p*-values ≤ 0.05, \*\* = *p*-values ≤ 0.01, \*\*\*\* = *p*-values ≤ 0.0001. OCR: oxygen consumption rate, FAs: Fatty acids, UF: unit fluorescence/well.


**Table 2.** Comparison of the mean values of key parameters for mitochondrial function (basal, MR, ATP production, and SRC) in myoblasts of patients (*n* = 5) and controls (*n* = 5) with or without 300 µM palmitate or oleate. Significant differences between patients and controls are shown if *p* ≤ 0.05.
