Scarpa M, Rigo A, Momo F, Isacchi G, Novelli G, Dallapiccola B
Biochem Biophys Res Commun. 1985 Jul 16;130(1):127-32. doi: 10.1016/0006-291x(85)90391-2.
The rate of generation of superoxide ion, the concentration of Cu, Zn superoxide dismutase and the hematological parameters were measured in red blood cells obtained from Fanconi anemia patients and from healthy individuals. No significative difference in the superoxide dismutase concentration was found, while the rate of generation of the superoxide ion doubled in Fanconi anemia patients. The steady-state concentration of the superoxide ion was calculated from these data and was found to be 2.3 times higher in Fanconi anemia erythrocytes than in controls. The possible consequences with respect to the alterations in FA are discussed.
测定了范可尼贫血患者和健康个体红细胞中超氧阴离子的生成速率、铜锌超氧化物歧化酶的浓度以及血液学参数。超氧化物歧化酶浓度未发现显著差异，而范可尼贫血患者中超氧阴离子的生成速率增加了一倍。根据这些数据计算了超氧阴离子的稳态浓度，发现范可尼贫血红细胞中的稳态浓度比对照组高2.3倍。讨论了这些改变对范可尼贫血可能产生的后果。
