title: Near normal levels of isoprenoid lipids in severe mevalonic aciduria.
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.
authors: Department of Biochemistry and Molecular Biology, University of South Florida College of Medicine, Tampa 33612.
authors: R K
authors: RK
authors: Keller
authors: W S
authors: WS
authors: Simonet
mesh_terms: 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
chemical_list: D004288:Dolichol Phosphates; D011106:Polyisoprenyl Phosphates; D013185:Squalene; D002784:Cholesterol; D006903:Hydroxymethylglutaryl CoA Reductases; D008798:Mevalonic Acid