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Various abnormalities in mitochondrial enzyme activities have been found, e.g. in myopathies. In only a restricted number of patients these disturbances can be demonstrated in fibroblasts. This restriction is caused by two facts: 1 some defects, found in muscle tissue, are not expressed in cultured fibroblasts and 2 some mitochondrial enzyme activities are hardly measurable in fibroblasts. Recently, chorionic villi have become important for detecting aberrations in, among others, enzyme activities. Using chorionic villi instead of amnion cells fixes the moment of antenatal diagnosis at an earlier tine. In the case of mitochondrial myopathies it is furthermore important that some enzyme activities can be measured in chorionic villi which can not be determined in fibroblasts. Among them are various oxidoreductases of the respiratory chain. Activity of cytochrome oxidase, pyruvate dehydrogenase complex and citrate synthase can be determined in both chorionic villi and fibroblasts. Control values (in mU/mg protein;mean± SD)in chorionic villi: The possibilities for antenatal diagnosis of mitochondrial myopathies have now clearly been extended.