出版社：American Society for Biochemistry and Molecular Biology
摘要：Patients with α-tocopherol transfer protein (α-TTP) defects experience neurological symptoms characteristic of vitamin E deficiency and depend on continuous high α-tocopherol supplements. We investigated the excretion of 2,5,7,8-tetramethyl-2(2′-carboxyethyl)-6-hydroxychroman (α-CEHC), a urinary metabolite of α-tocopherol, as a putative marker for the α-tocopherol status of α-TTP-deficient patients and control subjects. In three patients vitamin E supplementation was stopped for short periods of time, during which plasma α-tocopherol concentrations and urinary α-CEHC excretion were measured. In the patients, plasma α-tocopherol decreased below normal ( —Schuelke, M., A. Elsner, B. Finckh, A. Kohlschütter, C. Hübner, and R. Brigelius-Flohé. Urinary α-tocopherol metabolites in α-tocopherol transfer protein-deficient patients. J. Lipid Res. 2000. 41: 1543–1551.
关键词：α-tocopherol ; α-tocopherol metabolites ; α-CEHC ; α-tocopherol transfer protein ; AVED patients
