The Organelle Pathology and Demonstration of Mitochondrial Superoxide Dismutase Deficiency in Two Patients with Dubin–Johnson–Sprinz Syndrome

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.