ABSTRACT
Mutations of the α-tocopherol transfer protein (α-TTP) gene lead to reduced α-tocopherol concentrations in plasma and tissues that ultimately lead to a severe syndrome called ataxia with vitamin E deficiency (AVED) (1). Vitamin E therapy prevents progression of the syndrome and reverses some of the neurological symptoms (2,3). Vitamin E supplementation is also useful in a number of disorders, especially atherosclerosis, ischemic heart disease, and some cancers (4-6). The simple antioxidant function of vitamin E is not sufficient to explain all of the effects shown, and different biological roles have to be considered.
