Amyloidogenicity Of Ig Light Chains

Only a small proportion of Ig light chains are amy-loidogenic. For example, AL amyloidosis occurs in only about 12-15% of patients with myeloma.12 Certain structural features are related to amy-loiodogenicity: the X isotype and the VX6 variability subgroup.13 Two VX gene segments, 6a and 3r, seem to contribute to nearly 40% of the amyloidogenic X light chains.1415 There is some evidence that some of the amyloidogenic light chains undergo antigen-driven selection and mutations.14 Development of mutations in these proteins can then lead to destabilization of key structural domains and generation of an aggregation prone state.16 Improved understanding of the mechanisms of amyloidogenicity of the Ig light chains may allow the development of targeted therapies.

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