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Assessment of Tocopherol Metabolism and Oxidative Stress in Familial Hypobetalipoproteinemia

¹ Department of Core Clinical Pathology and Biochemistry, PathWest Laboratory Medicine WA, Royal Perth Hospital, Perth, WA, Australia.
² School of Medicine and Pharmacology, and ³ School of Surgery and Pathology, University of Western Australia. Crawley, WA, Australia.

^aAddress correspondence to this author at: Department of Core Clinical Pathology and Biochemistry, PathWest Laboratory Medicine WA, Royal Perth Hospital, GPO Box X2213, Perth, WA 6847, Australia. Fax 61 (08) 9224-1789; e-mail john.burnett{at}health.wa.gov.au.

Background: Vitamin E supplementation has been recommended for persons with familial hypobetalipoproteinemia (FHBL), a rare disorder of lipoprotein metabolism that leads to low serum -tocopherol and decreased LDL-cholesterol and apolipoprotein (apo) B. We examined the effect of truncated apoB variants on vitamin E metabolism and oxidative stress in persons with FHBL.

Methods: We studied 9 individuals with heterozygous FHBL [mean (SE) age, 40 (5) years; body mass index (BMI), 27 (10) kg/m²] and 7 normolipidemic controls [age, 41 (5) years; BMI, 25 (2) kg/m²]. We also studied 3 children—2 with homozygous FHBL (apoB-30.9) and 1 with abetalipoproteinemia—who were receiving -tocopherol supplementation. We used HPLC with electrochemical detection to measure - and -tocopherol in serum, erythrocytes, and platelets, and gas chromatography–mass spectrometry to measure F₂-isoprostanes and tocopherol metabolites in urine as markers of oxidative stress and tocopherol intake, respectively.

Results: Compared with controls, persons with FHBL had significantly lower fasting plasma concentrations of total cholesterol [2.4 (0.2) vs 4.7 (0.2) mmol/L], triglycerides [0.5 (0.1) vs 0.9 (0.1) mmol/L], LDL-cholesterol [0.7 (0.1) vs 2.8 (0.3) mmol/L], apoB [0.23 (0.02) vs 0.84 (0.08) g/L], -tocopherol [13.6 (1.0) vs 28.7 (1.4) µmol/L], and -tocopherol [1.0 (0.1) vs 1.8 (0.3) µmol/L] (all P <0.03). Erythrocyte -tocopherol was decreased [5.0 (0.2) vs 6.0 (0.3) µmol/L; P <0.005], but we observed no differences in lipid-adjusted serum tocopherols, erythrocyte -tocopherol, platelet - or -tocopherol, urinary F₂-isoprostanes, or tocopherol metabolites.

Conclusion: Taken together, our findings do not support the recommendation that persons with heterozygous FHBL receive vitamin E supplementation.

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				J. R. Burnett, S. Zhong, Z. G. Jiang, A. J. Hooper, E. A. Fisher, R. S. McLeod, Y. Zhao, P. H. R. Barrett, R. A. Hegele, F. M. van Bockxmeer, et al. Missense Mutations in APOB within the beta{alpha}1 Domain of Human APOB-100 Result in Impaired Secretion of ApoB and ApoB-containing Lipoproteins in Familial Hypobetalipoproteinemia J. Biol. Chem., August 17, 2007; 282(33): 24270 - 24283. [Abstract] [Full Text] [PDF]