Catalase in cultured skin fibroblasts from patients with the cerebro-hepato-renal (Zellweger) syndrome: normal maturation in peroxisome-deficient cells.

We have compared the properties of catalase in cultured skin fibroblasts from patients with the cerebro-hepato-renal (Zellweger) syndrome, in which peroxisomes are deficient, with those of catalase in fibroblasts from control subjects. The enzymes from the two types of fibroblasts are indistinguishable with respect to kinetic properties, subunit size and molecular mass of the native enzyme. The turnover of the enzyme, measured by following the rate of reappearance of catalase activity in fibroblasts after irreversible inactivation of existing molecules by 3-aminotriazole treatment of the cells, was the same in Zellweger fibroblasts as in control cells. These findings indicate that normal maturation of catalase can occur in the soluble cytoplasm and provide an explanation for the occurrence of extra-peroxisomal catalase in tissues and cells.