Comment: It is often useful to study diseases that are similar to gain further insight into each of them. As we have noted, dysfunctions in the Nrf2 system can exacerbate symptoms and influence the development of environmental disease and other health problems from Nrf2 deficiency have been associated with alterations in iron transport. 1 in 50,000 patients are effected by Friedrich's ataxia which is an inherited disorder that causes substantial neurological damage over a lifetime leading to early disability.
Background: Friedrich's ataxia (FA) is a condition that is the result of loss of mitochondrial frataxin leading to damage in a certain neurons. Recent studies show some of this is due to impairment in the Nrf2 signaling system. In Friedrich's ataxia, iron overload leads to damaged nerves and muscle cells.
A new study demonstrates one of the characteristics in FA is an increase in DNA damage. According to the head researcher, in FA "gene activity patterns that are associated with responses to DNA damage, and our comparisons and follow-up tests showed us that FRDA patients have far more damage than seen in healthy people." A better understanding of this disease should lead to better treatments.
Source: Excess DNA damage found in cells of patients with Friedreich's ataxia.
Pietsch, E. C., Chan, J. Y., Torti, F. M., and Torti, S. V. (2003). Nrf2 mediates the induction of ferritin h in response to xenobiotics and cancer chemopreventive dithiolethiones. Journal of Biological Chemistry, 278(4):2361-2369. http://www.citeulike.org/user/HEIRS/article/6543977
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