1. Liver biopsies from two patients with the Dubin—Johnson—Sprinz syndrome were subjected to analytical subcellular fractionation by sucrose-density-gradient centrifugation and enzymic micro-assays.

2. A selective deficiency of mitochondrial superoxide dismutase has been demonstrated in these patients.

3. The significance of this enzyme deficiency is discussed in relation to the organelle pathology of the syndrome.

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