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TitleRole of mutations and post-translational modifications in systemic AL amyloidosis studied by cryo-EM.
Journal, issue, pagesNat Commun, Vol. 12, Issue 1, Page 6434, Year 2021
Publish dateNov 5, 2021
AuthorsLynn Radamaker / Sara Karimi-Farsijani / Giada Andreotti / Julian Baur / Matthias Neumann / Sarah Schreiner / Natalie Berghaus / Raoul Motika / Christian Haupt / Paul Walther / Volker Schmidt / Stefanie Huhn / Ute Hegenbart / Stefan O Schönland / Sebastian Wiese / Clarissa Read / Matthias Schmidt / Marcus Fändrich /
PubMed AbstractSystemic AL amyloidosis is a rare disease that is caused by the misfolding of immunoglobulin light chains (LCs). Potential drivers of amyloid formation in this disease are post-translational ...Systemic AL amyloidosis is a rare disease that is caused by the misfolding of immunoglobulin light chains (LCs). Potential drivers of amyloid formation in this disease are post-translational modifications (PTMs) and the mutational changes that are inserted into the LCs by somatic hypermutation. Here we present the cryo electron microscopy (cryo-EM) structure of an ex vivo λ1-AL amyloid fibril whose deposits disrupt the ordered cardiomyocyte structure in the heart. The fibril protein contains six mutational changes compared to the germ line and three PTMs (disulfide bond, N-glycosylation and pyroglutamylation). Our data imply that the disulfide bond, glycosylation and mutational changes contribute to determining the fibril protein fold and help to generate a fibril morphology that is able to withstand proteolytic degradation inside the body.
External linksNat Commun / PubMed:34741031 / PubMed Central
MethodsEM (helical sym.)
Resolution3.1 Å
Structure data

12570

7nsl

EMDB-12570, PDB-7nsl:
AL amyloid fibril from a lambda 1 light chain
Method: EM (helical sym.) / Resolution: 3.1 Å

Source
  • homo sapiens (human)
  • Human (human)
KeywordsIMMUNE SYSTEM / amyloid / antibody / systemic amyloidosis / light chain

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