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Antioxid Redox Signal. 2008 Apr;10(4):837-41. doi: 10.1089/ars.2007.1874.

Can the therapeutic efficacy of tocotrienols in neurodegenerative familial dysautonomia patients be measured clinically?

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  • 1Laboratory for Familial Dysautonomia Research, Fordham University, Bronx, NY 10458, USA. rubin@fordham.edu

Abstract

Familial dysautonomia (FD) is an inherited, fatal, neurodegenerative disorder manifested by autonomic/hypertensive crises and cardiac instability. Patients produce little IKAP, the gene product of the affected mutated gene, and have low levels of monoamine oxidase A (MAO A), whose reduced presence appears to result in an increased accumulation of biogenic amines, which is a trigger for hypertensive crises. As ingestion of tocotrienols elevates IKAP and MAO A in FD patients, we examined their impact on the frequency of hypertensive crises and cardiac function. After 3 to 4 months of tocotrienol ingestion, approximately 80% of patients reported a significant (> or = 50%) decrease in the number of crises. In a smaller group of patients, a postexercise increase in heart rate and a decrease in the QT interval were observed in the majority of participants. Based on these findings, we hypothesize that tocotrienol therapy will improve the long-term clinical outlook and survival of individuals with FD.

PMID:
18177231
[PubMed - indexed for MEDLINE]
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