SUPEROXIDE DISMUTASE IN JUVENILE NEURONAL CEROID‐LIPOFUSCINOSIS (SPIELMEYER‐VOGT‐BATTEN'S DISEASE)

Abstract

Abstract— The ceroid‐lipofuscin pigments found in neuronal ceroid‐lipofuscinosis are believed to arise from lipid peroxidation‐damaged organelle membranes. In several in vitro systems superoxide dismutase inhibits lipid peroxidation. Erythrocytes and lymphocytes were prepared from ten patients with juvenile neuronal ceroid‐lipofuscinosis (Spielmeyer‐Vogt‐Batten's disease). Copper‐zinc and manganese superoxide dismutases were selectively assayed by a direct method on homogenates of the cells. No significant differences were found between the patients, neurological controls and healthy adults.