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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Symposium: Neurodegeneration with Brain Iron Accumulation
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Symposium: Neurodegeneration with Brain Iron Accumulation

机译：专题讨论会：神经变性与脑铁蓄积

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Aceruloplasminemia is characterized by progressive neurodegeneration with brain iron accumulation due to the complete lack of ceruloplasmin ferroxidase activity caused by mutations in the ceruloplasmin gene. Redox-active iron accumulation was found to be more prominent in the astrocytes than in the neurons. The most characteristic findings were abnormal or deformed astrocytes and globular structures of astrocytes. The lack of ceruloplasmin may primarily damage astrocytes in the aceruloplasminemic brains as a result of lipid peroxidation due to massive iron deposition. In the normal brain, iron may be continuously recycled between astrocytes and neurons, with transferrin acting as a shuttle. The glycosylphosphatidylinositol (GPI)-linked ceruloplasmin on astrocytes functions as a ferroxidase, mediating the oxidation of ferrous iron transported from the cytosol by ferroportin and its subsequent transfer to transferrin. In cases with aceruloplasminemia, neurons take up the iron from alternative sources of non-transferrin-bound iron, because astrocytes without GPI-linked ceruloplasmin cannot transport iron to transferrin. The excess iron in astrocytes could result in oxidative damage to these cells, and the neuronal cell protection offered by astrocytes would thus be disrupted. Neuronal cell loss may result from iron starvation in the early stage and from iron-mediated oxidation in the late stage. Ceruloplasmin may therefore play an essential role in neuronal survival in the central nervous system.
机译：铜蓝蛋白血症的特征是由于铜蓝蛋白基因突变引起的铜蓝蛋白铁氧合酶活性完全缺乏，导致进行性神经变性并伴有脑铁积聚。发现在星形胶质细胞中氧化还原活性铁积累比在神经元中更为显着。最典型的发现是星形胶质细胞异常或变形以及星形胶质细胞的球状结构。铜蓝蛋白的缺乏可能主要是由于大量铁沉积引起的脂质过氧化作用，从而损害了铜蓝蛋白脑中的星形胶质细胞。在正常的大脑中，铁可能在星形胶质细胞和神经元之间连续循环，而转铁蛋白则起穿梭作用。星形胶质细胞上与糖基磷脂酰肌醇（GPI）相连的铜蓝蛋白起铁氧化酶的作用，介导铁转运蛋白从细胞质中转运出的亚铁氧化并随后转移至运铁蛋白。在患有铜蓝蛋白血症的情况下，神经元会从非转铁蛋白结合的铁的其他来源吸收铁，因为没有GPI连接的铜蓝蛋白的星形胶质细胞无法将铁转运到铁蛋白。星形胶质细胞中过量的铁可能导致这些细胞的氧化损伤，因此星形胶质细胞提供的神经元细胞保护作用将被破坏。神经元细胞损失可能是由于早期的铁缺乏和后期的铁介导的氧化所致。因此，铜蓝蛋白在中枢神经系统的神经元存活中可能起重要作用。

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