Amyloid: From Starch to Finish.
Amyloid: From Starch to Finish.
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淀粉样蛋白：从淀粉到完成。
DOI：
10.1016/j.biopsych.2020.02.1182
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发表时间：
2020
影响因子：
10.6
通讯作者：
Mecca,AdamP
中科院分区：
文献类型：
--
作者：
Krystal,HannahL;Ross,DavidA;Mecca,AdamP
1639年，尼古拉斯·丰塔努斯（Nicholas Fontanus）检查了一个不寻常的标本：一个刚刚去世的年轻人，他的腹部充满了多余的液体，他的皮肤已经变成了严重黄疸的深黄色。在尸检过程中，Fontanus在他的脾脏中发现了大的白色结石。1722年，Jeremiah Wainewright解剖了一个病人，他的脖子肿胀，表明患有甲状腺疾病，但一个意想不到的发现挑战了诊断：在病人的胸腔下面，他的肝脏已经膨胀到正常大小的两到三倍，为一种神秘的粘土色物质腾出空间。1789年，Antoine Portal打开一位已故老妇人的尸体，发现了一个具有脂肪纹理的肝脏。很快，“猪油状”和“蜡状”的术语开始出现在医学教科书中，以描述患有这种奇怪疾病的肝脏和肾脏。最终，维尔茨堡巴伐利亚大学病理解剖学系主任鲁道夫·维尔肖（Rudolph Virchow）加入了进来。以研究细胞分裂而闻名的科学家魏尔啸不赞成这两种描述。他认为这种奇怪的物质是淀粉，一种通常在植物中发现的线性糖分子。但其他研究人员不同意，认为更准确的比较可能是纤维素，淀粉的纤维，难消化的表亲。于是，魏尔啸强行展开了这场辩论。在1854年，他应用了一个相对较新的术语-由德国植物学家马蒂亚斯·施莱登于1838年创建，用于描述正常的淀粉植物成分-用于沉积在人体组织中的奇怪物质（1）。这种神秘的物质仍然无法解释，但最终得到了一个统一的名字：淀粉样蛋白。
In 1639, Nicholas Fontanus examined an unusual specimen: a recently deceased young man whose abdomen sloshed with excess fluid and whose skin had turned the deep yellow of severe jaundice. During the autopsy, Fontanus found large white stones in his spleen. In 1722, Jeremiah Wainewright autopsied a patient whose swollen neck suggested thyroid disease, but an unexpected finding challenged the diagnosis: tucked underneath the patient’s ribcage, his liver had swelled to between two and three times its normal size to make room for a mysterious, clay-colored substance. In 1789, Antoine Portal opened the body of a deceased elderly woman to discover a liver with the texture of rendered fat.Soon, the terms “lardaceous” and “waxy” began appearing in medical textbooks to describe livers and kidneys suffering from this strange malady. Eventually, Rudolph Virchow, chair of pathologic anatomy at the University of Bavaria in Würzburg, weighed in. Virchow, a scientist most famous for his work on cell division, disapproved of both descriptors. He believed that the strange substance was starch, a linear sugar molecule typically found in plants. But other researchers disagreed, thinking that a more accurate comparison might be cellulose, starch’s fibrous, indigestible cousin. So Virchow forced the debate. In 1854, he applied a relatively new term–one created in 1838 by German botanist Matthias Schleiden to describe a normal, starchy plant component–to the strange material depositing in human tissues (1). The mysterious substance still defied explanation, but at last received a unifying name: amyloid.