Huang Y S, Marcel Y L, Vezina C, Barbeau A, Davignon J
Can J Neurol Sci. 1980 Nov;7(4):429-34. doi: 10.1017/s0317167100023015.
In a study of the fatty acid composition of erythrocyte membrane phospholipids in Friedreich's ataxia, a lower percentage of linoleic acid in phosphatidylcholine was demonstrated. An enzyme involving the exchange of lipids between plasma and erythrocyte membrane, lecithin: cholesteryl acyltransferase (LCAT) was also studied. It was found that the LCAT activity had a trend towards low values. However, crossing-over studies indicated that when the LCAT enzyme of patients was exposed to its own substrate it gave low activity values but that the result reverted to normal when control substrate was used.
在一项关于弗里德赖希共济失调患者红细胞膜磷脂脂肪酸组成的研究中,发现磷脂酰胆碱中亚油酸的百分比降低。还对一种涉及血浆与红细胞膜之间脂质交换的酶——卵磷脂胆固醇酰基转移酶(LCAT)进行了研究。发现LCAT活性有降低的趋势。然而,交叉研究表明,当患者的LCAT酶作用于自身底物时活性值较低,但使用对照底物时结果恢复正常。