Turnbull D M, Bartlett K, Watmough N J, Shepherd I M, Sherratt H S
Department of Neurology, University of Newcastle upon Tyne.
J Inherit Metab Dis. 1987;10 Suppl 1:105-12. doi: 10.1007/BF01812851.
Fatty acids are important substrates for resting and exercising skeletal muscle. Defects of fatty acid oxidation result in muscle pain and weakness, and there is often lipid accumulation in muscle fibres. Only a few of the several possible enzyme defects have been found and some of these have responded to therapy. Some techniques for the investigation of fatty acid oxidation are outlined.
脂肪酸是静息和运动状态下骨骼肌的重要底物。脂肪酸氧化缺陷会导致肌肉疼痛和无力，并且肌纤维中常出现脂质蓄积。在几种可能的酶缺陷中，仅发现了少数几种，其中一些对治疗有反应。本文概述了一些用于研究脂肪酸氧化的技术。
