Immunoglobulin aggregation leading to Russell body formation is prevented by the antibody light chain

Blood. 2010 Jan 14;115(2):282-8. doi: 10.1182/blood-2009-07-234864. Epub 2009 Oct 12.

Abstract

Russell bodies (RBs) are intracellular inclusions filled with protein aggregates. In diverse lymphoid disorders these occur as immunoglobulin (Ig) deposits, accumulating in abnormal plasma or Mott cells. In heavy-chain deposition disease truncated antibody heavy-chains (HCs) are found, which bear a resemblance to diverse polypeptides produced in Ig light-chain (LC)-deficient (L(-/-)) mice. In L(-/-) animals, the known functions of LC, providing part of the antigen-binding site of an antibody and securing progression of B-cell development, may not be required. Here, we show a novel function of LC in preventing antibody aggregation. L(-/-) mice produce truncated HC naturally, constant region (C)gamma and Calpha lack C(H)1, and Cmicro is without C(H)1 or C(H)1 and C(H)2. Most plasma cells found in these mice are CD138(+) Mott cells, filled with RBs, formed by aggregation of HCs of different isotypes. The importance of LC in preventing HC aggregation is evident in knock-in mice, expressing Cmicro without C(H)1 and C(H)2, which only develop an abundance of RBs when LC is absent. These results reveal that preventing antibody aggregation is a major function of LC, important for understanding the physiology of heavy-chain deposition disease, and in general recognizing the mechanisms, which initiate protein conformational diseases.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Gene Knock-In Techniques
  • Immunoglobulin Constant Regions / genetics
  • Immunoglobulin Constant Regions / metabolism*
  • Immunoglobulin Heavy Chains / genetics
  • Immunoglobulin Heavy Chains / metabolism*
  • Immunoglobulin Light Chains / genetics
  • Immunoglobulin Light Chains / metabolism*
  • Inclusion Bodies / genetics
  • Inclusion Bodies / metabolism*
  • Mice
  • Mice, Knockout
  • Plasma Cells / metabolism*
  • Plasma Cells / pathology
  • Syndecan-1 / genetics
  • Syndecan-1 / metabolism

Substances

  • Immunoglobulin Constant Regions
  • Immunoglobulin Heavy Chains
  • Immunoglobulin Light Chains
  • Sdc1 protein, mouse
  • Syndecan-1