重度甲羟戊酸尿症中类异戊二烯脂质水平接近正常。

Near normal levels of isoprenoid lipids in severe mevalonic aciduria.

作者信息

Keller R K, Simonet W S

机构信息

Department of Biochemistry and Molecular Biology, University of South Florida College of Medicine, Tampa 33612.

出版信息

Biochem Biophys Res Commun. 1988 Apr 29;152(2):857-61. doi: 10.1016/s0006-291x(88)80117-7.

DOI:10.1016/s0006-291x(88)80117-7

PMID:3365255

Abstract

The levels of cholesterol and dolichyl phosphate in the liver of an abortus with severe mevalonic aciduria were found to be approximately 60% of the mean of 5 age matched controls, while the level of squalene was within the normal range. Thus, despite a level of mevalonate kinase reported to be less than 1% of normal (Hoffmann, H. et al. (1986) N. Engl. J. Med. 314, 1610-1614), the liver was able to synthesize normal or near normal levels of isoprenoid lipids.

摘要

在患有严重甲羟戊酸尿症的流产胎儿肝脏中，胆固醇和磷酸多萜醇水平约为5名年龄匹配对照者均值的60%，而鲨烯水平在正常范围内。因此，尽管据报道甲羟戊酸激酶水平不到正常水平的1%（霍夫曼，H.等人（1986年）《新英格兰医学杂志》314卷，第1610 - 1614页），但肝脏仍能够合成正常或接近正常水平的类异戊二烯脂质。