Impaired ability of patients with familial isolated vitamin E deficiency to incorporate α-tocopherol into lipoproteins secreted by the liver

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DOIResolve DOI: http://doi.org/10.1172/JCI114452.
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TypeArticle
Journal titleJournal of Clinical Investigation
ISSN0021-9738
Volume85
Issue2
Pages397407; # of pages: 11
Subjectalpha tocopherol; chylomicron; high density lipoprotein; low density lipoprotein; radioisotope; very low density lipoprotein; adult; alpha tocopherol deficiency; article; blood level; case report; controlled study; female; human; lipoprotein metabolism; liver; male; priority journal; Absorption; Adult; Chylomicrons; Female; Human; Lipoproteins; Liver; Male; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.; Vitamin E; Vitamin E Deficiency
AbstractPlasma and lipoprotein α-tocopherol concentrations of four patients with familial isolated vitamin E deficiency and six control subjects were observed for 4 d after an oral dose (~15 mg) of RRR-α-tocopheryl acetate labeled with six deuterium atoms (d6-tocopherol). Chylomicron d6-tocopherol concentrations were similar in the two groups. d6-Tocopherol concentrations of plasma, very low (VLDL), low (LDL), and high (HDL) density lipoproteins were similar in the two groups only during the first 12 h; then these were significantly lower, and the rate of disappearance faster, in the patients. The times (t(max)) of the maximum chylomicron d6-tocopherol concentrations were similar for the two groups, but t(max) values in the controls increased in the order: chylomicrons < VLDL ≤ LDL ~ HDL, while the corresponding values in the patients were similar to the chylomicron t(max). Thus, plasma d6-tocopherol in controls increased during chylomicron and VLDL catabolism, whereas in patients it increased only during chylomicron catabolism, thereby resulting in a premature and faster decline in the plasma tocopherol concentration due to a lack of d6-tocopherol secretion from the liver. We suggest that these patients are lacking or have a defective liver 'tocopherol binding protein' that incroporates α-tocopherol into nascent VLDL.
Publication date
LanguageEnglish
AffiliationNational Research Council Canada (NRC-CNRC)
Peer reviewedYes
NPARC number21276684
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Record identifierc0bb3fa8-030e-4b43-b98c-76e39c9bd9ce
Record created2015-10-13
Record modified2016-05-09
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