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SUPEROXIDE DISMUTASE IN JUVENILE NEURONAL CEROID‐LIPOFUSCINOSIS (SPIELMEYER‐VOGT‐BATTEN'S DISEASE)
Author(s) -
Marklund S.,
Plum C. M.
Publication year - 1978
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1978.tb02667.x
Subject(s) - batten disease , lipofuscin , neuronal ceroid lipofuscinosis , superoxide dismutase , lipid peroxidation , lysosome , juvenile , dismutase , biochemistry , chemistry , endocrinology , biology , medicine , enzyme , gene , genetics
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.