老年系统性淀粉样变性中的原纤维源自正常的转甲状腺素蛋白。
Fibril in senile systemic amyloidosis is derived from normal transthyretin.
作者信息
Westermark P, Sletten K, Johansson B, Cornwell G G
机构信息
Department of Pathology, University of Linköping, Sweden.
出版信息
Proc Natl Acad Sci U S A. 1990 Apr;87(7):2843-5. doi: 10.1073/pnas.87.7.2843.
Abstract
The amyloid fibril in senile systemic amyloidosis (SSA), like that of familial amyloidotic polyneuropathy, is derived from transthyretin (TTR). SSA, however, is a common disease, affecting to some degree 25% of the population greater than 80 years old. In familial amyloidotic polyneuropathy, the amyloidogenesis has been considered to depend on point mutations leading to TTR variants. We show that the TTR molecule in SSA, on the other hand, has a normal primary structure. Factors other than the primary structure of TTR must therefore be important in the pathogenesis of TTR-derived amyloid.
摘要
与家族性淀粉样多神经病一样，老年系统性淀粉样变性（SSA）中的淀粉样纤维源自转甲状腺素蛋白（TTR）。然而，SSA是一种常见疾病，在80岁以上的人群中，约25%会受到不同程度的影响。在家族性淀粉样多神经病中，淀粉样变的发生被认为取决于导致TTR变体的点突变。另一方面，我们发现SSA中的TTR分子具有正常的一级结构。因此，除TTR一级结构外的其他因素在TTR衍生淀粉样变的发病机制中一定很重要。
相似文献
1
Fibril in senile systemic amyloidosis is derived from normal transthyretin.老年系统性淀粉样变性中的原纤维源自正常的转甲状腺素蛋白。
Proc Natl Acad Sci U S A. 1990 Apr;87(7):2843-5. doi: 10.1073/pnas.87.7.2843.
2
Amyloid fibril protein in familial amyloidotic polyneuropathy, Portuguese type. Definition of molecular abnormality in transthyretin (prealbumin).葡萄牙型家族性淀粉样多神经病中的淀粉样原纤维蛋白。转甲状腺素蛋白（前白蛋白）分子异常的定义。
J Clin Invest. 1984 Jul;74(1):104-19. doi: 10.1172/JCI111390.
3
Amyloid fibril composition and transthyretin gene structure in senile systemic amyloidosis.老年系统性淀粉样变中的淀粉样纤维成分与转甲状腺素蛋白基因结构
Lab Invest. 1995 Nov;73(5):703-8.
4
Systemic senile amyloidosis. Identification of a new prealbumin (transthyretin) variant in cardiac tissue: immunologic and biochemical similarity to one form of familial amyloidotic polyneuropathy.系统性老年性淀粉样变性。心脏组织中一种新的前白蛋白（转甲状腺素蛋白）变体的鉴定：与一种家族性淀粉样多神经病形式的免疫学和生化相似性。
J Clin Invest. 1989 Mar;83(3):836-43. doi: 10.1172/JCI113966.
5
Amyloid deposits in transthyretin-derived amyloidosis: cleaved transthyretin is associated with distinct amyloid morphology.转甲状腺素蛋白源性淀粉样变性中的淀粉样沉积物：裂解的转甲状腺素蛋白与独特的淀粉样形态相关。
J Pathol. 2005 Jun;206(2):224-32. doi: 10.1002/path.1759.
6
Transthyretin-derived senile systemic amyloidosis: clinicopathologic and structural considerations.转甲状腺素蛋白源性老年系统性淀粉样变性：临床病理及结构考量
Amyloid. 2003 Aug;10 Suppl 1:48-54.
7
Specific disruption of transthyretin(105-115) fibrilization using "stabilizing" inhibitors of transthyretin amyloidogenesis.使用“稳定”的转甲状腺素蛋白淀粉样变抑制剂特异性破坏转甲状腺素蛋白(105-115)纤维形成。
Biochemistry. 2012 Apr 24;51(16):3523-30. doi: 10.1021/bi3002727. Epub 2012 Apr 12.
8
Amyloid and nonfibrillar deposits in mice transgenic for wild-type human transthyretin: a possible model for senile systemic amyloidosis.野生型人转甲状腺素蛋白转基因小鼠中的淀粉样和非纤维状沉积物：老年系统性淀粉样变性的一种可能模型。
Lab Invest. 2001 Mar;81(3):385-96. doi: 10.1038/labinvest.3780246.
9
Clusterin regulates transthyretin amyloidosis.簇集素调节转甲状腺素蛋白淀粉样变性。
Biochem Biophys Res Commun. 2009 Oct 16;388(2):256-60. doi: 10.1016/j.bbrc.2009.07.166. Epub 2009 Aug 5.
10
Characterization of a transthyretin (prealbumin) variant associated with familial amyloidotic polyneuropathy type II (Indiana/Swiss).与II型家族性淀粉样多神经病（印第安纳/瑞士型）相关的转甲状腺素蛋白（前白蛋白）变体的特征分析
J Clin Invest. 1986 Oct;78(4):880-6. doi: 10.1172/JCI112675.
引用本文的文献
1
Initiation of transthyretin aggregation at neutral pH by fluid agitation.通过液体搅拌在中性pH条件下引发转甲状腺素蛋白聚集。
Proc Natl Acad Sci U S A. 2025 Mar 18;122(11):e2425230122. doi: 10.1073/pnas.2425230122. Epub 2025 Mar 11.
2
Misfolding of transthyretin in vivo is controlled by the redox environment and macromolecular crowding.体内转甲状腺素蛋白的错误折叠受氧化还原环境和大分子拥挤效应的控制。
J Biol Chem. 2025 Jan;301(1):108031. doi: 10.1016/j.jbc.2024.108031. Epub 2024 Nov 28.
3
Aggregation of Transthyretin by Fluid Agitation.通过液体搅拌使转甲状腺素蛋白聚集。
bioRxiv. 2024 Nov 11:2024.11.08.622726. doi: 10.1101/2024.11.08.622726.
4
Transthyretin amyloidosis prevalence and characteristics in Korean patients with heart failure with preserved or mildly reduced ejection fractions.转甲状腺素蛋白淀粉样变性在韩国射血分数保留或轻度降低心力衰竭患者中的患病率及特征。
Sci Rep. 2024 Oct 1;14(1):22863. doi: 10.1038/s41598-024-74191-0.
5
Assessment of transthyretin instability in patients with wild-type transthyretin amyloid cardiomyopathy.评估野生型转甲状腺素蛋白淀粉样心肌病患者的转甲状腺素蛋白不稳定性。
Sci Rep. 2024 Sep 3;14(1):20508. doi: 10.1038/s41598-024-71446-8.
6
Mispacking of the F87 sidechain drives aggregation-promoting conformational fluctuations in the subunit interfaces of the transthyretin tetramer.F87 侧链的包装错误导致转甲状腺素四聚体亚基界面中促进聚集的构象波动。
Protein Sci. 2024 Sep;33(9):e5101. doi: 10.1002/pro.5101.
7
Conformational Dynamics of an Amyloidogenic Intermediate of Transthyretin: Implications for Structural Remodeling and Amyloid Formation.淀粉样变相关转甲状腺素蛋白的构象动力学：对结构重塑和淀粉样形成的影响。
J Mol Biol. 2024 Aug 15;436(16):168673. doi: 10.1016/j.jmb.2024.168673. Epub 2024 Jun 21.
8
Identification of the interfacial regions in misfolded transthyretin oligomers.鉴定错误折叠转甲状腺素蛋白寡聚物的界面区域。
Biochim Biophys Acta Proteins Proteom. 2024 Sep 1;1872(5):141027. doi: 10.1016/j.bbapap.2024.141027. Epub 2024 May 23.
9
Thyroxine metabolite-derived 3-iodothyronamine (T1AM) and synthetic analogs as efficient suppressors of transthyretin amyloidosis.甲状腺素代谢产物衍生的3-碘甲状腺原氨酸（T1AM）及其合成类似物作为转甲状腺素蛋白淀粉样变性的有效抑制剂。
Comput Struct Biotechnol J. 2023 Sep 26;21:4717-4728. doi: 10.1016/j.csbj.2023.09.028. eCollection 2023.
10
Cardiac amyloidosis: evolving pathogenesis, multimodal diagnostics, and principles of treatment.心脏淀粉样变性：不断演变的发病机制、多模态诊断及治疗原则
EXCLI J. 2023 Aug 3;22:781-808. doi: 10.17179/excli2023-6284. eCollection 2023.
本文引用的文献
1
Senile cardiac amyloid is related to prealbumin.老年心脏淀粉样变与前白蛋白有关。
Scand J Immunol. 1980;12(6):503-6. doi: 10.1111/j.1365-3083.1980.tb00098.x.
2
Substrate specificity of a proteolytic enzyme isolated from a mutant of Pseudomonas fragi.从脆弱拟杆菌突变体中分离出的一种蛋白水解酶的底物特异性。
J Biol Chem. 1980 Feb 10;255(3):839-40.
3
Senile cardiac amyloid: evidence that fibrils contain a protein immunologically related to prealbumin.老年心脏淀粉样变：原纤维含有与前白蛋白免疫相关蛋白质的证据。
Immunology. 1981 Nov;44(3):447-52.
4
Amyloid fibril protein in familial amyloidotic polyneuropathy, Portuguese type. Definition of molecular abnormality in transthyretin (prealbumin).葡萄牙型家族性淀粉样多神经病中的淀粉样原纤维蛋白。转甲状腺素蛋白（前白蛋白）分子异常的定义。
J Clin Invest. 1984 Jul;74(1):104-19. doi: 10.1172/JCI111390.
5
Identification of amyloid prealbumin variant in familial amyloidotic polyneuropathy (Japanese type).家族性淀粉样多神经病（日本型）中淀粉样前白蛋白变异体的鉴定。
Biochem Biophys Res Commun. 1983 Nov 15;116(3):880-8. doi: 10.1016/s0006-291x(83)80224-1.
6
Frequency and distribution of senile cardiovascular amyloid. A clinicopathologic correlation.老年心血管淀粉样变的频率与分布：临床病理相关性
Am J Med. 1983 Oct;75(4):618-23. doi: 10.1016/0002-9343(83)90443-6.
7
Primary structure of an amyloid prealbumin and its plasma precursor in a heredofamilial polyneuropathy of Swedish origin.瑞典遗传性多神经病中淀粉样前清蛋白及其血浆前体的一级结构
Proc Natl Acad Sci U S A. 1984 Feb;81(3):694-8. doi: 10.1073/pnas.81.3.694.
8
Senile systemic amyloidosis.老年系统性淀粉样变
Am J Pathol. 1984 Dec;117(3):391-9.
9
Revised analysis of amino acid replacement in a prealbumin variant (SKO-III) associated with familial amyloidotic polyneuropathy of Jewish origin.与犹太裔家族性淀粉样多神经病相关的前白蛋白变体（SKO-III）中氨基酸置换的修订分析。
Biochem Biophys Res Commun. 1984 Sep 28;123(3):921-8. doi: 10.1016/s0006-291x(84)80222-3.
10
Primary structure of an amyloid prealbumin variant in familial polyneuropathy of Jewish origin.犹太裔家族性多神经病中一种淀粉样前白蛋白变体的一级结构。
Proc Natl Acad Sci U S A. 1983 Jan;80(2):539-42. doi: 10.1073/pnas.80.2.539.
Zotero 插件