Dissection of the amyloid formation pathway in AL amyloidosis
AL amyloidosis is caused by the accumulation of overproduced light chain (LC) fragments as fibrils in patient organs and it is the most prevalent systemic amyloidosis. Here, the authors combine biochemical and biophysical experiments to characterise the lag phase of a patient-derived truncated LC an...
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Nature Portfolio
2021
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oai:doaj.org-article:f5aabe6c6d2e492680e49c828972990f2021-11-14T12:34:06ZDissection of the amyloid formation pathway in AL amyloidosis10.1038/s41467-021-26845-02041-1723https://doaj.org/article/f5aabe6c6d2e492680e49c828972990f2021-11-01T00:00:00Zhttps://doi.org/10.1038/s41467-021-26845-0https://doaj.org/toc/2041-1723AL amyloidosis is caused by the accumulation of overproduced light chain (LC) fragments as fibrils in patient organs and it is the most prevalent systemic amyloidosis. Here, the authors combine biochemical and biophysical experiments to characterise the lag phase of a patient-derived truncated LC and they identify structural transitions that precede fibril formation.Pamina KazmanRamona M. AbsmeierHarald EngelhardtJohannes BuchnerNature PortfolioarticleScienceQENNature Communications, Vol 12, Iss 1, Pp 1-10 (2021) |
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Science Q Pamina Kazman Ramona M. Absmeier Harald Engelhardt Johannes Buchner Dissection of the amyloid formation pathway in AL amyloidosis |
description |
AL amyloidosis is caused by the accumulation of overproduced light chain (LC) fragments as fibrils in patient organs and it is the most prevalent systemic amyloidosis. Here, the authors combine biochemical and biophysical experiments to characterise the lag phase of a patient-derived truncated LC and they identify structural transitions that precede fibril formation. |
format |
article |
author |
Pamina Kazman Ramona M. Absmeier Harald Engelhardt Johannes Buchner |
author_facet |
Pamina Kazman Ramona M. Absmeier Harald Engelhardt Johannes Buchner |
author_sort |
Pamina Kazman |
title |
Dissection of the amyloid formation pathway in AL amyloidosis |
title_short |
Dissection of the amyloid formation pathway in AL amyloidosis |
title_full |
Dissection of the amyloid formation pathway in AL amyloidosis |
title_fullStr |
Dissection of the amyloid formation pathway in AL amyloidosis |
title_full_unstemmed |
Dissection of the amyloid formation pathway in AL amyloidosis |
title_sort |
dissection of the amyloid formation pathway in al amyloidosis |
publisher |
Nature Portfolio |
publishDate |
2021 |
url |
https://doaj.org/article/f5aabe6c6d2e492680e49c828972990f |
work_keys_str_mv |
AT paminakazman dissectionoftheamyloidformationpathwayinalamyloidosis AT ramonamabsmeier dissectionoftheamyloidformationpathwayinalamyloidosis AT haraldengelhardt dissectionoftheamyloidformationpathwayinalamyloidosis AT johannesbuchner dissectionoftheamyloidformationpathwayinalamyloidosis |
_version_ |
1718429204306984960 |