A Study of Saturated Very Long Chain Fatty Acid in Skin Fibroblast and Plasma of Adrenoleukodystrophy Patients

腎上腺白質退化症患者皮膚纖維芽細胞及血漿中飽和長鍵脂防酸之研究

林清淵(Ching-Yuang Lin)

《Acta Paediatrica Sinica》 26卷5期 (1985/10) Pp. 405-410

摘要

5位診斷為腎上腺白質退化症患者均切取一小片皮膚以皮膚纖維芽細胞培養技術增養出皮廣織維芽細胞後，再將細胞打破利用氣液色層分析法定量這5位患者皮膚纖維芽細胞內及其血漿中飽和長鍵脂肪酸之量。結果發現在腎上腺白質退化症患者之皮膚纖維芽細胞及血漿中26個碳之長鍵飽和脂肪酸之量分別為正常對照組之6倍及4倍。26個碳與24個碳飽和長鍵脂肪酸之量之比在腎上腺白質退化症患者血漿中亦大約為正常對照組之6倍。以上結果表示利用上述之檢查技術可作為腎上腺白質退化症之一診斷工具。

並列摘要

Gas-liquid chromatography and skin fibroblast culture were used to measure the saturated, very long chain fatty acids in the plasma and cultured skin fibroblasts from five patients with adrenoleukodystrophy (ALD). The total hexacosanoate (C26:0) contents of ALD fibroblasts and of plasma were sixfold and fourfold higher, respectively, than normal values. The C26:0 to C22:0 plasma ratios were also about sixfold higher than normal. Thus, these techniques can serve as parameters for diagnosis of ALD.

並列關鍵字

adrenoleukodystrophy ； hexacosanoate (26:0) ； very long-chain fatty acid