Steve Brown wrote:
>
> The FA gene produces too little of the protein Frataxin, which normally
> inhibits the over-production of iron in an energy-producing part of the
> human cell called the mitochondria. This shortage leads to a toxic
> buildup of iron (iron overload). The iron reacts with oxygen to produce
> free radicals, toxic and highly reactive substances that kill the
> mitochondria and compromise cell metabolism. Eventually, cells shut down
> and die from oxidative damage.. Current research suggests that
> antioxidants may help to reduce or eliminate this damage.
>
--
While I am not familiar with iron overload as a pathogenesis for FA (a
disease I know little about in the first place), if it is a factor why
not remove the toxic iron just as in hemochromatosis (with which I am
more familiar) and not have to worry about free radicals and
anti-oxidants? Each therapeutic phlebotomy of 500 cc gets rid of 250 mg
Fe, monitor ferratin levels till overload is gone - if Fe is a factor,
this sounds as reasonable as treating the effects of excess Fe. (Mind
you, I am *not* advocating bleeding and leaches for Rx of FA! Since I
don't remember any hepatic sequelae with FA I wouldn't think the common
picture of an iron overload disease corresponds to FA in any case.)
????
Phil
Philip G. Dunlap, D.O., MPH, MPM, PhD.
Pew Doctoral Fellow, Heller School, Brandeis University
4 Bailey Hill Road
Natick, MA, 01760, USA
[log in to unmask]
(508) 650-9097
(508) 650-9152 - fax
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