An ataxia telangiectasia model: inefficient cell differentiation and possible reversal by serine protease inhibitors, tumor necrosis factor inhibitors, dexamethasone, and glutathione enhancers

Abstract 

Ataxia telangiectasia (AT) is a rare genetic disorder. Symptoms of the disease include cerebellar ataxia, depressed immunoresponsiveness, increased sensitivity to radiation, and leukemias. Various kinds of AT cells show reduced efficiency of differentiation. The ataxia telangiectasia gene (ATM) may reduce differentiation by suppressing cell responsivity to insulin. Insulin sensitivity seems lower in AT. Tumor necrosis factor may overactivate NF-kappa B in AT, and this increases the radiosensitivity of AT cells. Intracellular reduced glutathione may also become depleted. The reduced levels of glutathione may further alter differentiation of AT cells. Serine protease inhibitors may counteract the effects of tumor necrosis factor. Glutathione enhancers may also prove valuable as therapy.

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