Journal
BRAIN RESEARCH
Volume 1201, Issue -, Pages 167-176Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.brainres.2008.01.044
Keywords
ataxia; brain; gender; liver; transgenic mice; tocopherol transfer protein; vitamin E
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Funding
- NIEHS NIH HHS [R01 ES011985-03, R01 ES011985-02, R01 ES011985-01A2, R01 ES011985, ES 011985-02, ES 011985] Funding Source: Medline
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Ataxia with vitamin E deficiency is caused by mutations in a-tocopherol transfer protein (alpha-TTP) gene and it can be experimentally generated in mice by alpha-TTP gene inactivation (alpha-TTP-KO). This study compared alpha-tocopherol (alpha-T) concentrations of five brain regions and of four peripheral organs from 5 months old, male and female, wild-type (WT) and alpha-TTP-KO mice. All brain regions of female WT mice contained significantly higher alpha-T than those from WT males. alpha-T concentration in the cerebellum was significantly lower than that in other brain regions of WT mice. These sex and regional differences in brain alpha-T concentrations do not appear to be determined by alpha-TTP expression which was undetectable in an brain regions. All the brain regions of alpha-TTP-KO mice were severely depleted in alpha-T. The concentration of another endogenous antioxidant, total glutathione, was unaffected by gender but was decreased slightly but significantly in most brain regions of alpha-TTP-KO mice. The results show that both gender and the hepatic a-TTP, but not brain alpha-TTP gene expression are important in determining alpha-T concentrations within the brain. interestingly, functional abnormality (ataxia) develops only very late in alpha-TTP-KO mice in spite of the severe alpha-tocopherol deficiency in the brain starting at an early age. (C) 2008 Elsevier B.V. All rights reserved.
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