Residual Catalase in Japanese Type Acatalasemia

抄録

Acatalasemia and hypocatalasemia are genetic diseases due to defective or incomplete catalase activity in blood cells and, in some cases, in tissue cells, but acatalasemic cells have some residual catalase. Half of the patients may suffer from severe oral gangrene but rest have no pathologic symptoms. Cells from the patients have glutathione peroxidase and may havenearly normal levels of other hydrogen peroxide decomposing enzymes. These enzymes together with residual catalase may protect the cells from damage from excess hydrogen peroxide, though this has not been verified. The biochemical characteristics of the residual catalases differ among patients from different areas and from those of animals, e.g. the catalase from the Japanese type acatalasemia is similar to that of a normal person, whereas the residual catalases in the Swiss type acatalasemia and the radiation induced mouse acatalasemia differ from those of normal individuals.