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DOI | 10.1038/s41467-017-00197-0 |
Cryo-EM structure of a transthyretin-derived amyloid fibril from a patient with hereditary ATTR amyloidosis | |
Schmidt, Matthias1; Wiese, Sebastian2; Adak, Volkan1; Engler, Jonas1; Agarwal, Shubhangi3; Fritz, Guenter3,4; Westermark, Per5; Zacharias, Martin6; Faendrich, Marcus1 | |
2019-11-01 | |
发表期刊 | NATURE COMMUNICATIONS |
ISSN | 2041-1723 |
出版年 | 2019 |
卷号 | 10 |
文章类型 | Article |
语种 | 英语 |
国家 | Germany; Sweden |
英文摘要 | ATTR amyloidosis is one of the worldwide most abundant forms of systemic amyloidosis. The disease is caused by the misfolding of transthyretin protein and the formation of amyloid deposits at different sites within the body. Here, we present a 2.97 angstrom cryo electron microscopy structure of a fibril purified from the tissue of a patient with hereditary Val30Met ATTR amyloidosis. The fibril consists of a single protofilament that is formed from an N-terminal and a C-terminal fragment of transthyretin. Our structure provides insights into the mechanism of misfolding and implies the formation of an early fibril state from unfolded transthyretin molecules, which upon proteolysis converts into mature ATTR amyloid fibrils. |
领域 | 资源环境 |
收录类别 | SCI-E |
WOS记录号 | WOS:000493712700001 |
WOS关键词 | SYNCHROTRON X-RAY ; RECONSTRUCTION ; REFINEMENT ; VARIANTS ; CLEAVAGE ; CORE |
WOS类目 | Multidisciplinary Sciences |
WOS研究方向 | Science & Technology - Other Topics |
URL | 查看原文 |
引用统计 | |
文献类型 | 期刊论文 |
条目标识符 | http://119.78.100.173/C666/handle/2XK7JSWQ/203401 |
专题 | 资源环境科学 |
作者单位 | 1.Ulm Univ, Inst Prot Biochem, D-89081 Ulm, Germany; 2.Ulm Univ, Core Unit Mass Spectrometry & Prote, D-89081 Ulm, Germany; 3.Univ Hohenheim, Inst Microbiol, D-70599 Stuttgart, Germany; 4.Univ Freiburg, Fac Med, Inst Neuropathol, D-79106 Freiburg, Germany; 5.Uppsala Univ, Dept Immunol Genet & Pathol, SE-75185 Uppsala, Sweden; 6.Tech Univ Munich, Dept Phys, D-85748 Garching, Germany |
推荐引用方式 GB/T 7714 | Schmidt, Matthias,Wiese, Sebastian,Adak, Volkan,et al. Cryo-EM structure of a transthyretin-derived amyloid fibril from a patient with hereditary ATTR amyloidosis[J]. NATURE COMMUNICATIONS,2019,10. |
APA | Schmidt, Matthias.,Wiese, Sebastian.,Adak, Volkan.,Engler, Jonas.,Agarwal, Shubhangi.,...&Faendrich, Marcus.(2019).Cryo-EM structure of a transthyretin-derived amyloid fibril from a patient with hereditary ATTR amyloidosis.NATURE COMMUNICATIONS,10. |
MLA | Schmidt, Matthias,et al."Cryo-EM structure of a transthyretin-derived amyloid fibril from a patient with hereditary ATTR amyloidosis".NATURE COMMUNICATIONS 10(2019). |
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