Modeling immunoglobulin light chain amyloidosis in Caenorhabditis elegans.

ABSTRACT: Cardiomyopathy determines the prognosis of patients with immunoglobulin light chain (AL) amyloidosis, a rare systemic disease caused by the misfolding and deposition of monoclonal light chains (LCs). The reasons underlying their cardiac tropism remain unknown, and an animal model recapitulating the main pathological features of AL amyloidosis is needed. Taking advantage of the similarities between the vertebrate cardiac muscle and Caenorhabditis elegans pharynx, we developed a new transgenic nematode expressing a human amyloidogenic λ LC, the sequence of which was deduced from a patient with AL amyloidosis with cardiac involvement (MNH). Strains expressing a non-amyloidogenic LC (MNM) or the empty vector only (MNV) were generated as controls. At variance with controls, LCs expressed in the

SUBMITTER: Romeo M 

PROVIDER: S-EPMC12320968 | biostudies-literature | 2025 Jul

REPOSITORIES: biostudies-literature