Kieseier B C, Wisniewski K E, Schuller-Levis G, Park E, Goebel H H
Department of Pathological Neurobiology, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, New York, USA.
Neuropediatrics. 1996 Aug;27(4):202-3. doi: 10.1055/s-2007-973788.
The neuronal ceroid-lipofuscinoses (NCL) are a group of inherited progressive encephalopathies. Striking histomorphological feature of the NCL is the accumulation of storage material within the lysosomes in neural and extraneural cells. The basic underlying defect causing the disease is not known. Presupposing a disturbance in lipid peroxidation, some authors recommend antioxidant treatment to slow down the progression of the disease. In this study, the superoxide radical production of polymorphonuclear leukocytes as one potential source of reactive oxygen species was measured in this disorder for the first time. No significant difference in this production between affected individuals and healthy controls could be detected. Our findings cast doubt on the therapeutic benefit of antioxidant treatment.
神经元蜡样脂褐质沉积症（NCL）是一组遗传性进行性脑病。NCL显著的组织形态学特征是神经细胞和神经外细胞的溶酶体内蓄积储存物质。导致该疾病的根本缺陷尚不清楚。基于脂质过氧化存在紊乱的假设，一些作者建议采用抗氧化治疗以减缓疾病进展。在本研究中，首次对这种疾病中作为活性氧潜在来源之一的多形核白细胞的超氧自由基生成进行了测量。未检测到患病个体与健康对照之间在这种生成上有显著差异。我们的研究结果使人对抗氧化治疗的疗效产生怀疑。
