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DanLewis said:

I have a question for the scientists out there.

A friend of mine suffers from Friedreich's Ataxia. Friedreich's Ataxia (FA) is a neurological disorder caused by a mutation in the FXN gene, the gene which is ordinarily responsible for the induction of the protein frataxin within the mitochondria. The inability to produce sufficient levels of frataxin is thought to adversely effect the functioning of the mitochondria in several ways. One theory is that insufficient frataxin inhibits the activity of manganese superoxide dismutase  (MnSOD).  As I understand it, MnSOD is one of the primary proteins involved in cellular defense against oxidative stress. MnSOD reacts with reactive oxygen species (ROS), such as superoxide and peroxide, and converts them into more stable compounds, such as hydrogen peroxide.   Without sufficient activity levels of MnSOD to effectively detoxify such ROS, the ROS react with cell structures, DNA, lipid, proteins, and enzymes, adversely effecting their normal functioning or causing cell death.

Recently I was told that resveratrol might offer some relief to FA patients, but the person who told me did not explain how it might help.  I did some research and found a 2007 study by Ellen L. Robb, Melissa M. Page, Brent E. Wiens, Jeffrey A. Stuart, entitled "Molecular mechanisms of oxidative stress resistance induced by resveratrol: Specific and progressive induction of MnSOD". That study found that two weeks exposure to resveratrol increased MnSOD protein level 6-fold and activity 14-fold in normal human lung fibroblasts cells.

My question is three fold:  Can anyone confirm that the induction of MnSOD would be the way resveratrol might help an FA patient? Is it accepted science that resveratrol induces the expression or activity of MnSOD, and if so, dose anyone know of any work attempting to determine if that induction of MnSOD is dependent upon normal levels of mitochondrial frataxin? If it is dependent, my friend is out of luck, because he has very low levels of mitochondrial frataxin.

There is a whole other issue regarding the safety of increasing the MnSOD activity in the presence of high levels of mitochondrial iron, a phenomena found in FA patients, but that is a question for another day, if anyone is interested.

Any insights would be greatly appreciated.

I have a question for the scientists out there.

A friend of mine suffers from Friedreich's Ataxia. Friedreich's Ataxia (FA) is a neurological disorder caused by a mutation in the FXN gene, the gene which is ordinarily responsible for the induction of the protein frataxin within the mitochondria. The inability to produce sufficient levels of frataxin is thought to adversely effect the functioning of the mitochondria in several ways. One theory is that insufficient frataxin inhibits the activity of manganese superoxide dismutase  (MnSOD).  As I understand it, MnSOD is one of the primary proteins involved in cellular defense against oxidative stress. MnSOD reacts with reactive oxygen species (ROS), such as superoxide and peroxide, and converts them into more stable compounds, such as hydrogen peroxide.   Without sufficient activity levels of MnSOD to effectively detoxify such ROS, the ROS react with cell structures, DNA, lipid, proteins, and enzymes, adversely effecting their normal functioning or causing cell death.

Recently I was told that resveratrol might offer some relief to FA patients, but the person who told me did not explain how it might help.  I did some research and found a 2007 study by Ellen L. Robb, Melissa M. Page, Brent E. Wiens, Jeffrey A. Stuart, entitled "Molecular mechanisms of oxidative stress resistance induced by resveratrol: Specific and progressive induction of MnSOD". That study found that two weeks exposure to resveratrol increased MnSOD protein level 6-fold and activity 14-fold in normal human lung fibroblasts cells.

My question is three fold:  Can anyone confirm that the induction of MnSOD would be the way resveratrol might help an FA patient? Is it accepted science that resveratrol induces the expression or activity of MnSOD, and if so, dose anyone know of any work attempting to determine if that induction of MnSOD is dependent upon normal levels of mitochondrial frataxin? If it is dependent, my friend is out of luck, because he has very low levels of mitochondrial frataxin.

There is a whole other issue regarding the safety of increasing the MnSOD activity in the presence of high levels of mitochondrial iron, a phenomena found in FA patients, but that is a question for another day, if anyone is interested.

Any insights would be greatly appreciated.