Oxidative stress is always associated with Friedreich’s Ataxia (FRDA) also accompained by impaired mitochondrial functions. Patients are currently treated with idebenone, a CoQ10 analogue, believed effective in view of its ability to counteract free radical damages. Taking into account that efficacy of Vit. E on oxidative stress related pathologies is well documented and considering our esperience in the field of the class of natural vitamin E “tocotrienol” we have started the present investigation in order to develop a model useful to investigate the efficacy of a tocotrienol based approaches on oxidative stress damage protection. A suitable natural cocktail of enantiomerically pure tocotrienols (alpha, beta, gamma and delta from palm oil) has been selected and tested in patients monitoring several biochemical parameters. Blood samples from five young FRDA patients who assumed tocotrienol (5 mg/kg/day), for two months. The following features were studied: white blood cell gene expression of SOD-1, SOD-2, catalase, GPX-1, GSR and GSTM-1; plasma content of GSH and GSSG; plasma Oxygen Radical Absorbance Capacity; amount of plasma carbonylated proteins; urinary levels of Hexanoyl-Lysine adduct; lipid composition of erythrocyte membranes. Such wide array of different markers consistently pointed to the presence of oxidative stress in FRDA patients, despite the fact that the idebenone therapy had not been discontinued. However, even a two month low-dose tocotrienol supplementation led to the decrease of oxidative stress indexes and to parameter values that approached those of healthy controls. Moreover, there are evidences that a longer tocotrienol treatment may be more effective in reducing oxidative stress.
DEVELOPMENT OF NOVEL APPROACHES TO THE COMPLEMENTARY THERAPY OF FRIEDREICH ATAXIA: ORAL TOCOTRIENOL SUPPLEMENTATION DECREASE ASSOCIATED OXIDATIVE STRESS
MANFREDINI, Stefano;VERTUANI, Silvia;MALISARDI, Gemma;
2011
Abstract
Oxidative stress is always associated with Friedreich’s Ataxia (FRDA) also accompained by impaired mitochondrial functions. Patients are currently treated with idebenone, a CoQ10 analogue, believed effective in view of its ability to counteract free radical damages. Taking into account that efficacy of Vit. E on oxidative stress related pathologies is well documented and considering our esperience in the field of the class of natural vitamin E “tocotrienol” we have started the present investigation in order to develop a model useful to investigate the efficacy of a tocotrienol based approaches on oxidative stress damage protection. A suitable natural cocktail of enantiomerically pure tocotrienols (alpha, beta, gamma and delta from palm oil) has been selected and tested in patients monitoring several biochemical parameters. Blood samples from five young FRDA patients who assumed tocotrienol (5 mg/kg/day), for two months. The following features were studied: white blood cell gene expression of SOD-1, SOD-2, catalase, GPX-1, GSR and GSTM-1; plasma content of GSH and GSSG; plasma Oxygen Radical Absorbance Capacity; amount of plasma carbonylated proteins; urinary levels of Hexanoyl-Lysine adduct; lipid composition of erythrocyte membranes. Such wide array of different markers consistently pointed to the presence of oxidative stress in FRDA patients, despite the fact that the idebenone therapy had not been discontinued. However, even a two month low-dose tocotrienol supplementation led to the decrease of oxidative stress indexes and to parameter values that approached those of healthy controls. Moreover, there are evidences that a longer tocotrienol treatment may be more effective in reducing oxidative stress.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
