3.1.3. Fatty Acid Oxidation (FAO)

The Difference between Patients and the Controls

Independent of the applied substrate (oleate or palmitate), MR, ATP-R, and SRC were similar in patients and controls at 37 ◦C and 40 ◦C. BR was higher in controls than in patients, however, only at 37 ◦C and in presence of palmitate (Figure 2, Table 4). The increase of temperature from 37 ◦C to 40 ◦C led to a significant decrease in all key parameters (BR, MR, ATP-R, and SRC) in both, patients and controls (Table 5).

≤ **Figure 2.** Evaluation of ß-oxidation using a Seahorse XF96 Cell Analyzer in myoblasts from patients (*n* = 5) and controls (*n* = 5). The measurements were performed at 37 ◦C or 40 ◦C as described in methods. (**A**) basal respiration (BR), (**B**) maximal respiration (MR), (**C**) ATP linked respiration (ATP-R), and (**D**) spare respiratory capacity (SRC) by use of palmitate or oleate as substrate. BR, MR, ATP-R, and SRC (key parameters) were significantly reduced upon increased temperature from 37 ◦C to 40 ◦C in both patients and controls (significance shown in Table 5) \* = *p*-values ≤ 0.05. OCR: oxygen consumption rate, UF: unit fluorescence/well.

**Table 4.** Measurement of ß-oxidation using a Seahorse XF96 Cell Analyzer in myoblasts of patients (*n* = 5) and controls (*n* = 5) with palmitate or oleate as substrate at 37 ◦C and 40 ◦C. Significance was set at *p* = 0.05. FAO: fatty acid oxidation.


**Table 4.** *Cont.*


**Table 5.** Evaluation of ß-oxidation using a Seahorse XF96 Cell Analyzer in myoblasts of patients (*n* = 5) and controls (*n* = 5) with palmitate or oleate as substrate. The difference between values obtained at 37 ◦C and 40 ◦C is shown if the results were significant (*p*-values ≤ 0.05).


The Difference between Palmitate and Oleate

The important parameters BR, MR, ATP-R, and SRC were mostly similar with palmitate or oleate as substrate. Only SRC was higher in controls in the presence of oleate as substrate compared to the value resulted from palmitate as substrate at 37 ◦C (Table 6).

**Table 6.** Evaluation of ß-oxidation using a Seahorse XF96 Cell Analyzer at 37 ◦C and 40 ◦C in myoblasts of patients (*n* = 5) and controls (*n* = 5) with palmitate or oleate as substrate. The difference between values obtained using palmitate or oleate as substrate is shown if the results were significant (*p*-values ≤ 0.05), Pa: palmitate. O: oleate.

