Changes in nerve excitability indices in hereditary transthyretin amyloidosis
Changes in nerve excitability indices in hereditary transthyretin amyloidosis
复制标题
遗传性转甲状腺素蛋白淀粉样变性神经兴奋性指标的变化
DOI：
10.1080/13506129.2019.1582480
复制
发表时间：
2019
期刊：
影响因子：
5.5
通讯作者：
Ando Yukio
中科院分区：
文献类型：
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作者：
Yamashita Taro;Ueda Mitsuharu;Misawa Sonoko;Inoue Yasuteru;Masuda Teruaki;Misumi Yohei;Takamatsu Kotaro;Obayashi Konen;Kuwabara Satoshi;Ando Yukio
遗传性转甲状腺素（ATTR, ATTRm）淀粉样变性，又称转甲状腺素型家族性淀粉样多神经病变（atr - fap），是由突变型转甲状腺素（TTR）引起的常染色体显性系统性淀粉样变性，危及生命[文献1]。虽然多神经病变的轴突变性型是ATTRm淀粉样变的主要表现之一，但轴突的病理生理变化尚未得到很好的研究。最近开发了一种半自动程序来阐明各种神经兴奋性指数，称为QTRAC[引文2-4]。本研究旨在探讨ATTRm淀粉样变性的轴突离子病理生理机制。
BackgroundHereditary transthyretin (ATTR, ATTRm) amyloidosis, which is also called transthyretin-type familial amyloid polyneuropathy (ATTR-FAP), is a life-threatening, autosomal dominant systemic amyloidosis caused by mutant transthyretin (TTR)[Citation 1]. Although the axonal degeneration type of polyneuropathy is one of the primary manifestations of ATTRm amyloidosis, pathophysiological changes in the axons have not been well investigated. A semiautomatic program to elucidate various nerve excitability indices called QTRAC has recently been developed [Citation 2–4]. This study aimed to explore the axonal ionic pathophysiology in ATTRm amyloidosis.