Residual Catalase in Japanese Type Acatalasemia

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Abstract
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 others have no pathologic symptoms. Cells from affected patients have glutathione peroxidase and may have nearly normal levels of other H2O2 although 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.

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