Huntington' disease (HD) is an autosomal dominant neurodegenerative disease characterized by progressive motor, psychiatric, and cognitive deterioration. HD is, together with spinocerebellar ataxias, spinobulbar muscular atrophy and dentatorubral-pallido- luysian atrophy, one of the nine disorders caused by an expansion of glutamine residues in the causative protein where the polyglutamine expansion cause aberrant protein folding. Since an excessive metal's accumulation in organs may induce protein misfolding and oxidative stress, we have studied the blood concentration of essential (Cr, Co, Cu, Fe, Mn, Mo, Ni, Se, Zn) and nonessential (As, Cd, Sb, Sn, V) trace elements in HD patients. We found increased levels of the essential elements iron, chromium, selenium and zinc and of the nonessential element arsenic in the blood of HD patients. Since alteration in metals homeostasis may contribute to the pathogenesis of neurodegenerative disease and could eventually constitute a target for therapy, we may suggest the utilize of the blood metal profile as a further in vivo tool to study and characterize Huntington disease.

中文翻译：

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亨廷顿舞蹈病患者血液中的微量元素分布。

亨廷顿舞蹈病（HD）是一种常染色体显性遗传神经退行性疾病，其特征在于进行性运动，精神病和认知能力下降。HD与脊髓小脑性共济失调，脊髓小球性肌萎缩和齿下肌-丘脑-卢氏肌萎缩一起，是由病原蛋白中谷氨酰胺残基膨胀引起的九种疾病之一，其中聚谷氨酰胺膨胀导致异常蛋白折叠。由于过多的金属在器官中的积累可能会引起蛋白质错误折叠和氧化应激，因此我们研究了必需（Cr，Co，Cu，Fe，Mn，Mo，Ni，Se，Zn）和非必需（As，Cd，Sb）的血液浓度，Sn，V）中的微量元素。我们发现HD患者血液中铁，铬，硒和锌等必需元素和非必需砷的含量增加。