Miyajima H, Fujimoto M, Kohno S, Kaneko E, Gitlin J D
First Department of Medicine, Hamamatsu University School of Medicine, Japan.
Neurology. 1998 Oct;51(4):1188-90. doi: 10.1212/wnl.51.4.1188.
Aceruloplasminemia is a disorder of iron metabolism characterized by degeneration of the retina and basal ganglia. CSF from affected patients showed a threefold increased iron concentration that was associated with increased superoxide dismutase activity and lipid peroxidation products. These findings support the hypothesis that iron-mediated lipid peroxidation contributes to neurodegeneration in patients with aceruloplasminemia. Such measurements may have value in assessing disease progression as well as the results of iron chelation and other therapeutic interventions.
无铜蓝蛋白血症是一种铁代谢紊乱疾病，其特征为视网膜和基底神经节变性。患病患者的脑脊液中铁浓度增加了两倍，这与超氧化物歧化酶活性增加和脂质过氧化产物增多有关。这些发现支持了这样一种假说，即铁介导的脂质过氧化作用导致了无铜蓝蛋白血症患者的神经变性。此类测量对于评估疾病进展以及铁螯合和其他治疗干预的效果可能具有价值。
