Mitochondrial function is altered in horse atypical myopathy

作者：Lemieux Helene; Boemer Francois; van Galen Gaby; Serteyn Didier; Amory Helene; Baise Etienne; Cassart Dominique; van Loon Gunther; Marcillaud Pitel Christel; Votion Dominique M^*

来源：Mitochondrion, 2016, 30: 35-41.

DOI：10.1016/j.mito.2016.06.005

• Horse
• Acer
• Methylenecyclopropyl acetic acid-CoA
• Skeletal muscle
• Rhabdomyolysis
• Acylcarnitines
• High-resolution respirometry

Equine atypical myopathy in Europe is a fatal rhabdomyolysis syndrome that results from the ingestion of hypoglycin A contained in seeds and seedlings of Acer pseudoplatanus (sycamore maple). Acylcamitine concentrations in serum and muscle OXPHOS capacity were determined in 15 atypical myopathy cases. All but one acylcarnitine were out of reference range and mitochondrial respiratory capacity was severely decreased up to 49% as compared to 10 healthy controls. The hallmark of atypical myopathy thus consists of a severe alteration in the energy metabolism including a severe impairment in muscle mitochondrial respiration that could contribute to its high death rate.

• 出版日期2016-9