What is the role of vitamin E in the pathophysiology of abetalipoproteinemia (ABL)?

Updated: Mar 06, 2018
  • Author: Vibhuti N Singh, MD, MPH, FACC, FSCAI; Chief Editor: George T Griffing, MD  more...
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Answer

Most of the clinical symptoms of ABL are the result of defects in the absorption and transport of vitamin E. Normally, vitamin E is transported from the intestine to the liver, where it is repackaged and incorporated into the assembling VLDL particle by the tocopherol-binding protein. In the circulation, VLDL is converted to LDL, and vitamin E is transported by LDL to peripheral tissues and delivered to cells via the LDL receptor. Patients with ABL are markedly deficient in vitamin E because of the deficient plasma transport of vitamin E, which requires hepatic secretion of apoB-containing lipoproteins. Most of the major clinical symptoms, especially those of the nervous system and retina, are primarily due to vitamin E deficiency. This hypothesis is supported by the fact that other disorders involving vitamin E deficiency are characterized by similar symptoms and pathologic changes. [11]


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