Susuki Seiko, Ando Yukio, Sato Takashi, Nishiyama Masami, Miyata Masanori, Suico Mary Ann, Shuto Tsuyoshi, Kai Hirofumi
Department of Molecular Medicine, Graduate School of Pharmaceutical Sciences, Global COE Cell Fate Regulation Research and Education Unit, Kumamoto University, 5-1 Oe-Honmachi, Kumamoto, Japan.
Amyloid. 2008 Jun;15(2):108-16. doi: 10.1080/13506120802006013.
There is accumulating evidence of the involvement of biological metal imbalance in the progression of amyloid diseases such as Alzheimer's, Parkinson's and prion diseases. However, the mineral status in patients affected with familial amyloidotic polyneuropathy (FAP) has not been investigated. It is the aim of this study to determine the metal concentrations in the serum and in the transthyretin (TTR) amyloid fibrils of FAP amyloidogenic TTR (ATTR) V30M patients. Multi-elemental analysis of 17 metals by high-resolution inductively coupled plasma mass spectrometry (ICP-MS) revealed a significant decrease of the metals Fe, Cu, Zn, Cs and Ba in the serum of FAP patients (mean age 38.5 +/- 8.3 years; duration of disease 4 +/- 2.6 years) in comparison with that of healthy individuals (mean age 36.2 +/- 9.2 years). On the other hand, these metals, except Cs, were found at high levels in the amyloid fibrils of FAP patients (mean age 55.8 +/- 9.2; duration of disease 6.5 +/- 1.3 years) compared with other metals. These findings firstly suggest that the mineral status could be a candidate factor, which participates in the wide spectrum of clinical pictures of FAP patients.
越来越多的证据表明，生物金属失衡与阿尔茨海默病、帕金森病和朊病毒病等淀粉样疾病的进展有关。然而，家族性淀粉样多神经病（FAP）患者的矿物质状况尚未得到研究。本研究的目的是测定FAP淀粉样前体蛋白转甲状腺素（TTR）V30M患者血清和TTR淀粉样纤维中的金属浓度。通过高分辨率电感耦合等离子体质谱（ICP-MS）对17种金属进行多元素分析发现，与健康个体（平均年龄36.2±9.2岁）相比，FAP患者（平均年龄38.5±8.3岁；病程4±2.6年）血清中的铁、铜、锌、铯和钡等金属显著减少。另一方面，与其他金属相比，除铯外，这些金属在FAP患者（平均年龄55.8±9.2岁；病程6.5±1.3年）的淀粉样纤维中含量较高。这些发现首先表明，矿物质状况可能是一个参与FAP患者广泛临床症状的候选因素。
