Bence Jones proteins: a powerful tool for the fundamental study of protein chemistry and pathophysiology

Bence Jones proteins are typically found in patients with monoclonal plasma cell or related B-cell immunoproliferative disorders, i.e., multiple myeloma, light-chain-associated amyloidosis (amyloidosis AL), and, occasionally, benign gammopathy, lymphoma, and leukemia. Because of their monoclonal origin and resulting chemical homogeneity, as well as their ready availability, much of the fundamental information on immunoglobulin structure came initially from analysis of Bence Jones proteins and light chains isolated from monoclonal immunoglobulins. The interactions at the interface of the V sub(L) dimer are a function of primary structure and solution conditions. Study of these interactions has led to new information on the relationship between protein structure and function and, as discussed here, can account for specific pathophysiological properties associated with Bence Jones proteins.