A new isoleucine substitution of Val-20 in transthyretin tetramers selectively impairs dimer-dimer contacts and causes systemic amyloidosis

Dieter E. Jenne; Klaus Denzel; Petra Blatzinger

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A new isoleucine substitution of Val-20 in transthyretin tetramers selectively impairs dimer-dimer contacts and causes systemic amyloidosis

机译：运甲状腺素蛋白四聚体中Val-20的新异亮氨酸替代选择性地损害二聚体-二聚体接触并引起系统性淀粉样变性

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The most frequent form of inherited amy- loidoses is associated with mutations in the transthyretin (TTR) gene coding for 127-amino acid residues of four iden- tical, noncovalently linked subunits that form a pair of dimers in the plasma protein complex. Amyloid fibrils containing the variant and to a lesser extent the wild-type form of the TTR molecule are deposited in various organs, including periph- eral nerves and the myocardium, with polyneuropathy and cardiomyopathy as major clinical manifestations.

机译：遗传淀粉样糖的最常见形式与运甲状腺素蛋白（TTR）基因中的突变有关，后者编码四个同构的，非共价连接的亚基的127个氨基酸残基，这些亚基在血浆蛋白复合物中形成一对二聚体。含有变体和较小程度的TTR分子野生型形式的淀粉样蛋白原纤维沉积在各种器官中，包括周围神经和心肌，多发性神经病和心肌病是主要的临床表现。

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《Proceedings of the National Academy of Sciences of the United States of America》 |1996年第13期|p.6302-6307|共6页
作者
Dieter E. Jenne; Klaus Denzel; Petra Blatzinger;
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收录信息美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
原文格式 PDF
正文语种 eng
中图分类自然科学总论;
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1. The electrocardiographic features associated with cardiac amyloidosis of variant transthyretin isoleucine 122 type in Afro-Caribbean patients [J] . DunguJ., SattianayagamP.T., WhelanC.J., The American heart journal . 2012,第1期

机译：非裔加勒比患者变甲状腺素蛋白异亮氨酸122型变异与心脏淀粉样变性相关的心电图特征
2. The transthyretin cDNA sequence is normal in transthyretin‐derived senile systemic amyloidosis [J] . Betsholtz C., Christmanson L., Gustavsson Aring, FEBS Letters . 1991,第1a2期

机译：运甲状腺素蛋白引起的老年系统性淀粉样变性患者的运甲状腺素蛋白cDNA序列正常
3. Approach to diagnosis in systemic amyloidosis: initial findings and time from symptoms onset to diagnosis (light-chain amyloidosis vs. transthyretin). Problems and observations. [J] . Di Girolamo M, Monno D, Pirro MR, Amyloid: the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis . 2011,第Suppla1期

机译：系统性淀粉样变性的诊断方法：从症状发作到诊断的初步发现和时间（轻链淀粉样变性与运甲状腺素蛋白）。问题和观察。
4. COMPARISON OF CARDIAC AMYLOIDOSIS ASSOCIATED WITH WILD TYPE TRANSTHYRETIN AND THE ISOLEUCINE 122 VARIANT [C] . H.J. Lachmann, H.J.B.. Goodman, J.D. Gillmore, International Symposium on Amyloidosis . 2008

机译：与野生型Transthyretin和异吲哚122变体相关的心脏淀粉样蛋白病的比较
5. The role of transthyretin cysteine modification in senile systemic amyloidosis. [D] . Kingsbury, Jonathan S. 2007

机译：运甲状腺素蛋白半胱氨酸修饰在老年系统性淀粉样变性中的作用。
6. A new isoleucine substitution of Val-20 in transthyretin tetramers selectively impairs dimer-dimer contacts and causes systemic amyloidosis. [O] . D E Jenne, K Denzel, P Blätzinger, 1996

机译：运甲状腺素蛋白四聚体中Val-20的新异亮氨酸替代选择性地损害二聚体-二聚体接触并引起系统性淀粉样变性。
7. A new isoleucine substitution of Val-20 in transthyretin tetramers selectively impairs dimer-dimer contacts and causes systemic amyloidosis. [O] . Jenne, D E, Denzel, K, Blätzinger, P, 1996

机译：运甲状腺素蛋白四聚体中Val-20的新异亮氨酸替代选择性地损害二聚体-二聚体接触并引起系统性淀粉样变性。

A new isoleucine substitution of Val-20 in transthyretin tetramers selectively impairs dimer-dimer contacts and causes systemic amyloidosis

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