Near normal levels of isoprenoid lipids in severe mevalonic aciduria.
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.
Keller
R K
RK
Department of Biochemistry and Molecular Biology, University of South Florida College of Medicine, Tampa 33612.
Simonet
W S
WS
D002784:Cholesterol / Q000097:blood; D004288:Dolichol Phosphates / Q000378:metabolism*; D005260:Female; D005315:Fetal Diseases / Q000378:metabolism; D006801:Humans; D006903:Hydroxymethylglutaryl CoA Reductases / Q000378:metabolism; D008099:Liver / Q000378:metabolism; D008297:Male; D008661:Metabolism, Inborn Errors / Q000378:metabolism*; D008798:Mevalonic Acid / Q000652:urine*; D011106:Polyisoprenyl Phosphates / Q000378:metabolism*; D011247:Pregnancy; D013185:Squalene / Q000378:metabolism
D004288:Dolichol Phosphates; D011106:Polyisoprenyl Phosphates; D013185:Squalene; D002784:Cholesterol; D006903:Hydroxymethylglutaryl CoA Reductases; D008798:Mevalonic Acid