[Gamma-1 heavy chain disease with the demonstration of Bence-Jones proteins].

Abstract

We describe a 61-year-old patient suffering from gamma-1-heavy-chain disease (gamma 1-HCD) associated with Bence-Jones-lambda proteinemia and proteinuria. The analysis of the patients gamma 1-HCD protein (WIN) shows a deletion of the complete Fd fragment. The N-terminal seven amino-acid residue does not resemble any of the known immunoglobulin-heavy-chain variable regions. Unexpectedly, in PBL-DNA and in DNA from EBV-immortalized cells we found in addition to the expected predominantly rearranged Ig-lambda-light-chain gene a predominant rearrangement of an Ig-kappa gene. These findings show that the gamma-1-heavy-chain disease of the patient involves a defective regulation of Ig-light-chain-gene activation as well.

• Authors: G Hauke, U Krawinkel, E Schiltz, B Metz, A Hollmann, H H Peter
• Journal: Immunitat und Infektion (Immun Infekt) Vol. 19 Issue 3 Pg. 89-90 (Jun 1991) Germany
• Vernacular Title: Gamma-1-Schwerkettenkrankheit mit Nachweis von Bence-Jones-Proteinen.
• PMID: 1916871 (Publication Type: Case Reports, English Abstract, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Immunoglobulin gamma-Chains
• Immunoglobulin kappa-Chains
• Immunoglobulin lambda-Chains
• Bence Jones Protein

Topics

• Amino Acid Sequence
• Bence Jones Protein (analysis, urine)
• Gene Rearrangement
• Heavy Chain Disease (blood, urine)
• Humans
• Immunoelectrophoresis
• Immunoglobulin gamma-Chains (chemistry)
• Immunoglobulin kappa-Chains (genetics)
• Immunoglobulin lambda-Chains (genetics)
• Male
• Middle Aged
• Molecular Sequence Data