对正常和丙酮酸脱氢酶缺乏的培养人成纤维细胞的代谢研究。
Metabolic studies on normal and pyruvate dehydrogenase deficient cultured human fibroblasts.
作者信息
Borud O, Stroomme J H
出版信息
Scand J Clin Lab Invest. 1977 Sep;37(5):419-23.
Secondary metabolic derangements may occur in cultured fibroblasts with a defined enzyme deficiency. The metabolism of cells deficient of pyruvate dehydrogenase (5% of normal) has been studied using radioactive labelled substrates. Compared to normal control cells the activity of glycolysis was 149% (P less than 0.001), pentose phosphate shunt 144% (P less than 0.01), citric acid cycle 80% (P less than 0.002), and oxidation of acetate was 30% (P less than 0.01). The oxidation of palmitate and octanoate were not significantly different from that of control cells. Metabolic studies on fibroblasts may serve as a useful screening procedure for the detection of enzyme defects, but the results should be cautiously interpreted with respect to the localization of the primary defect.
在具有特定酶缺陷的培养成纤维细胞中可能会发生继发性代谢紊乱。已使用放射性标记底物研究了丙酮酸脱氢酶缺乏（为正常水平的5%）的细胞的代谢情况。与正常对照细胞相比，糖酵解活性为149%（P<0.001），磷酸戊糖途径为144%（P<0.01），柠檬酸循环为80%（P<0.002），乙酸氧化为30%（P<0.01）。棕榈酸和辛酸的氧化与对照细胞无显著差异。对成纤维细胞的代谢研究可作为检测酶缺陷的有用筛查程序，但对于原发性缺陷的定位，应谨慎解释结果。
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