Cytotoxicity of amyloidogenic immunoglobulin light chains in cell culture.

ABSTRACT: Light-chain amyloidosis (AL) is a devastating protein-misfolding disease characterized by abnormal proliferation of plasma cells in the bone marrow that secrete monoclonal immunoglobulin light chains that misfold and form amyloid fibrils, thus causing organ failure and death. Numerous reports on different protein-misfolding diseases show that soluble oligomeric species populated by amyloidogenic proteins can be quite toxic to cells. However, it is not well established whether the soluble immunoglobulin light-chain species found in circulation in patients with AL are toxic to cells in target organs. We determined the cellular toxicity of two well-characterized light-chain variable domain proteins from cardiac AL patients and their corresponding germline protein, devoid of somatic mutations.

SUBMITTER: Sikkink LA 

PROVIDER: S-EPMC3032327 | biostudies-literature | 2010 Nov

REPOSITORIES: biostudies-literature