Das A K, Holmes R D, Wilson G N, Hajra A K
Mental Health Research Institute, University of Michigan, Ann Arbor 48104.
Lipids. 1992 Jun;27(6):401-5. doi: 10.1007/BF02536379.
Chronic feeding of 1-O-octadecyl-sn-glycerol (batyl alcohol) to patients suffering from congenital deficiency in tissue ether glycerolipids showed an increase in the plasmalogens content of their erythrocytes. However, nothing is known about the ether lipid content of other tissues in these patients. Feeding 1-O-heptadecyl-sn-glycerol to young rats showed that this uncommon ether lipid was incorporated to a high extent into the plasmalogens of all tissues except brain. Comparative studies with other precursors, such as 3-O-heptadecyl-sn-glycerol, heptadecanol and heptadecanoic acid, indicated a stereospecific incorporation of the dietary 1-O-alkyl-sn-glycerols into tissue plasmalogens without cleavage of the ether bond. Dietary ether lipids were also shown to be transferred from mothers to suckling rats, but not from pregnant rats to fetuses. The implication of these results to possible dietary ether lipid therapy for patients suffering from peroxisomal disorders is discussed.
对患有组织醚甘油脂质先天性缺乏症的患者长期喂食1-O-十八烷基-sn-甘油(鲨肝醇),结果显示其红细胞中缩醛磷脂含量增加。然而,对于这些患者其他组织中的醚脂质含量却一无所知。对幼鼠喂食1-O-十七烷基-sn-甘油的研究表明,这种不常见的醚脂质在很大程度上被整合到除大脑外所有组织的缩醛磷脂中。与其他前体(如3-O-十七烷基-sn-甘油、十七烷醇和十七烷酸)的对比研究表明,膳食中的1-O-烷基-sn-甘油以立体特异性方式整合到组织缩醛磷脂中,且醚键未发生断裂。膳食醚脂质还被证明可从母体转移至哺乳幼鼠,但不会从怀孕大鼠转移至胎儿。本文讨论了这些结果对于过氧化物酶体疾病患者可能的膳食醚脂质治疗的意义。