An example of a neurodegenerative disorder that is believed to be caused by free-radicals. I find the last line of this press release very encouraging since I have some friends who have children with Ataxia-Telangiectasia. It also gives me hope for other friends with Multiple System Atrophy/Shy-Drager, OPCA, Friedreich's Ataxia and Spinocerebellar ataxia. Regards, Pam >August 17, 1999 > >Antioxidants May Fight Rare Disease > >.c The Associated Press > > By LAURAN NEERGAARD > >AP Medical Writer > >WASHINGTON (AP) - Animal research suggests doctors might look to >antioxidants to help fight a rare but lethal inherited disease that puts >most child sufferers in wheelchairs by their teens and kills them by their >20s. > >The new research is only in mice, not in people, but it indicates that a >buildup of damage from destructive molecules called ``free radicals'' may >help fuel the deadly disease called ataxia-telangiectasia - pronounced >Ay-TACK-see-uh Teh-LAN-jick-TAY-sha - and better known as A-T. > >The A-T Children's Project, a patient advocacy group that helps fund A-T >research, considers the finding a strong enough lead that it will gather >antioxidant experts in November to determine which substances should be >tested as possible A-T treatments. > >``Now we have absolutely no treatment for this brutal disease,'' said >project director Brad Margus, who has two sons with A-T. The new research >``gives us impetus to proceed with pursuing the most ... powerful >antioxidants we can try, first on mice and hopefully on children with the >disease.'' > >A-T's first signs are an unsteady gait, slurred speech and poor muscle >coordination in young children. Eventually, children lose all motor >control. A-T also weakens their immune systems. Patients usually die in >their 20s of cancer or infections. > >About 500 American children have A-T, which worldwide strikes about one in >every 40,000 children. > >The genetic defect that causes A-T was discovered in 1995, but that >discovery has not yet led to treatments. > >The new research, from California's Salk Institute for Biological Studies, >suggests the gene defect leaves A-T patients without protection against >damage from the free radicals that may kill their brain cells. > >Everybody experiences this so-called oxidative damage from free radicals, >byproducts of metabolism that attack cells. Most people's bodies can >counter and repair oxidative damage, helped by antioxidants like vitamins C >and E. But sometimes free radicals go out of control, causing or >contributing to cancer, Alzheimer's and other diseases. > >Salk lead researcher Dr. Carrolee Barlow bred mice with the A-T gene defect >and examined cells from the cerebellum, the brain region that controls >movement. A-T patients' progressive loss of motor control occurs when vital >cerebellum cells die. > >In this week's Proceedings of the National Academy of Sciences, Barlow >reports on evidence that suggests the mouse brain cells were killed by free >radicals. > >Some desperate A-T families already have tried over-the-counter >antioxidants with no apparent effect, scientists note. > >``It may be that those compounds just aren't strong enough,'' said Barlow, >who is testing in mice an experimental antioxidant created by the National >Cancer Institute. > >Other researchers, Margus said, are creating antioxidants that promise to >be hundreds of times more powerful than Vitamin C. > >AP-NY-08-17-99 0215EDT