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Open AccessArticle

Palmitate but Not Oleate Exerts a Negative Effect on Oxygen Utilization in Myoblasts of Patients with the m.3243A>G Mutation: A Pilot Study

1
Department of Neurology, Martin-Luther-University Halle-Wittenberg, 06120 Halle (Saale), Germany
2
Center for Basic Medical Research, Martin-Luther-University Halle-Wittenberg, 06120 Halle (Saale), Germany
3
Department of Neurology, Ulm University, 89070 Ulm, Germany
*
Author to whom correspondence should be addressed.
Life 2020, 10(9), 204; https://doi.org/10.3390/life10090204
Received: 5 August 2020 / Revised: 4 September 2020 / Accepted: 15 September 2020 / Published: 16 September 2020
(This article belongs to the Special Issue Impaired Mitochondrial Bioenergetics under Pathological Conditions)
It is known that exposure to excess saturated fatty acids, especially palmitate, can trigger cellular stress responses interpreted as lipotoxicity. The effect of excessive free fatty acids on oxidative phosphorylation capacity in myoblasts of patients with the m.3243A>G mutation was evaluated with the mitochondrial (Mito) stress test using a Seahorse XF96 analyzer. ß-oxidation, measured with the Seahorse XF96 analyzer, was similar in patients and controls, and reduced in both patients and controls at 40 °C compared to 37 °C. Mito stress test in the absence of fatty acids showed lower values in patients compared to controls. The mitochondrial activity and ATP production rates were significantly reduced in presence of palmitate, but not of oleate in patients, showing a negative effect of excessive palmitate on mitochondrial function in patients. Diabetes mellitus is a frequent symptom in patients with m.3243A>G mutation. It can be speculated that the negative effect of palmitate on mitochondrial function might be related to diacylglycerols (DAG) and ceramides (CER) mediated insulin resistance. This might contribute to the elevated risk for diabetes mellitus in m.3243A>G patients. View Full-Text
Keywords: fatty acid oxidation; palmitate; oleate; m.3243A> G mutation fatty acid oxidation; palmitate; oleate; m.3243A>; G mutation
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MDPI and ACS Style

Motlagh Scholle, L.; Schieffers, H.; Al-Robaiy, S.; Thaele, A.; Lehmann Urban, D.; Zierz, S. Palmitate but Not Oleate Exerts a Negative Effect on Oxygen Utilization in Myoblasts of Patients with the m.3243A>G Mutation: A Pilot Study. Life 2020, 10, 204. https://doi.org/10.3390/life10090204

AMA Style

Motlagh Scholle L, Schieffers H, Al-Robaiy S, Thaele A, Lehmann Urban D, Zierz S. Palmitate but Not Oleate Exerts a Negative Effect on Oxygen Utilization in Myoblasts of Patients with the m.3243A>G Mutation: A Pilot Study. Life. 2020; 10(9):204. https://doi.org/10.3390/life10090204

Chicago/Turabian Style

Motlagh Scholle, Leila; Schieffers, Helena; Al-Robaiy, Samiya; Thaele, Annemarie; Lehmann Urban, Diana; Zierz, Stephan. 2020. "Palmitate but Not Oleate Exerts a Negative Effect on Oxygen Utilization in Myoblasts of Patients with the m.3243A>G Mutation: A Pilot Study" Life 10, no. 9: 204. https://doi.org/10.3390/life10090204

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