van Laack H L, Ruitenbeek W, Trijbels J M, Sengers R C, Gabreëls F J, Janssen A J, Kerkhof C M
Department of Pediatrics, University Hospital, University of Nijmegen, The Netherlands.
Clin Chim Acta. 1988 Jan 15;171(1):109-18. doi: 10.1016/0009-8981(88)90296-3.
Pyruvate dehydrogenase (E1) catalyzes the rate-limiting step of the pyruvate dehydrogenase complex. Since E1 activity of human muscle tissue is low, a sensitive method is needed for diagnostic purposes. Measurement of 14CO2 production from [1-14C]pyruvate provides a specific and sensitive assay for measuring E1 activity. We use as artificial electron acceptor dichlorophenolindophenol (DCPIP) instead of the often applied ferricyanide. The method can be applied to small muscle samples obtained by needle or open biopsy. We prefer to use total homogenate because E1 activities in homogenate are higher than in the corresponding 600-g supernatant of skeletal muscle tissue. Control values in homogenate are higher or of the same order as those reported by others.
丙酮酸脱氢酶（E1）催化丙酮酸脱氢酶复合体的限速步骤。由于人体肌肉组织的E1活性较低，因此需要一种灵敏的方法用于诊断目的。测量[1-14C]丙酮酸生成的14CO2可提供一种特异性且灵敏的测定E1活性的方法。我们使用二氯酚靛酚（DCPIP）作为人工电子受体，而非常用的铁氰化物。该方法可应用于通过针吸活检或开放活检获取的小肌肉样本。我们更倾向于使用总匀浆，因为匀浆中的E1活性高于骨骼肌组织相应的600g上清液中的活性。匀浆中的对照值高于或与其他研究报道的值处于同一水平。
