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  • Title: Expanded CTG repeats in myotonin protein kinase increase susceptibility to oxidative stress.
    Author: Usuki F, Ishiura S.
    Journal: Neuroreport; 1998 Jul 13; 9(10):2291-6. PubMed ID: 9694217.
    Abstract:
    The effect of oxidative stress on myogenic cells with expanded CTG repeats in the myotonin protein kinase (MtPK) gene was investigated using MtPK cDNA-transformants in order to investigate the disease process underlying myotonic dystrophy. We employed methylmercury as a model for reagents that produce reactive oxygen species (ROS). Mutant MtPK cDNA transformants containing 46 CTG repeats treated with 1 microM methylmercury for 24 h underwent cell death showing the characteristics of apoptosis. In contrast, methylmercury-induced cytotoxicity was weaker in wild type MtPK cDNA transformants. Antioxidants such as N-acetyl-L-cysteine and trolox suppressed methylmercury-induced apoptosis, indicating that the intracellular generation of ROS plays an important role. These studies suggest that expanded CTG repeats in MtPK increase the susceptibility of cells to oxidative stress.
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