Marklund S L, Santavuori P, Westermarck T
Clin Chim Acta. 1981 Oct 26;116(2):191-8. doi: 10.1016/0009-8981(81)90022-x.
The neuronal ceroid-lipofuscinoses are characterized by a widespread deposit in the body of pigments believed to be end products of lipid peroxidation damaged organelles. CuZn superoxide dismutase, Mn superoxide dismutase, catalase and glutathione peroxidase, enzymes which conceivably might protect against lipid peroxidation, were investigated in blood cells from patients afflicted with infantile, late infantile and juvenile neuronal ceroid-lipofuscinosis. In some cases the enzymic activities were slightly lower than the activities of the controls, but no deficiencies which might be of etiolgical importance were revealed.
神经元蜡样脂褐质沉积症的特征是在体内广泛沉积一种色素，该色素被认为是脂质过氧化损伤细胞器的终产物。对患有婴儿型、晚婴儿型和青少年型神经元蜡样脂褐质沉积症患者的血细胞中铜锌超氧化物歧化酶、锰超氧化物歧化酶、过氧化氢酶和谷胱甘肽过氧化物酶进行了研究，这些酶理论上可能具有防止脂质过氧化的作用。在某些情况下，酶活性略低于对照组，但未发现可能具有病因学重要性的缺陷。
