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AL amyloid fibril from a lambda 3 light chain

ABSTRACT:

SUBMITTER: Marcus Fändrich

PROVIDER: EMPIAR-10457 | biostudies-other |

REPOSITORIES: biostudies-other

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Cryo-EM structure of a light chain-derived amyloid fibril from a patient with systemic AL amyloidosis.

Radamaker Lynn L Lin Yin-Hsi YH Annamalai Karthikeyan K Huhn Stefanie S Hegenbart Ute U Schönland Stefan O SO Fritz Günter G Schmidt Matthias M Fändrich Marcus M

Nature communications 20190320 1

Amyloid fibrils derived from antibody light chains are key pathogenic agents in systemic AL amyloidosis. They can be deposited in multiple organs but cardiac amyloid is the major risk factor of mortality. Here we report the structure of a λ1 AL amyloid fibril from an explanted human heart at a resolution of 3.3 Å which we determined using cryo-electron microscopy. The fibril core consists of a 91-residue segment presenting an all-beta fold with ten mutagenic changes compared to the germ line. Th ...[more]

PMID: 30894526

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