PMID- 7680298 OWN - NLM STAT- MEDLINE DCOM- 19930405 LR - 20190512 IS - 0009-9104 (Print) IS - 1365-2249 (Electronic) IS - 0009-9104 (Linking) VI - 91 IP - 3 DP - 1993 Mar TI - Primary structure of a variable region of the V kappa I subgroup (ISE) in light chain deposition disease. PG - 506-9 AB - Although structural abnormalities of monoclonal immunoglobulin light chains (LC) are suspected to play a determinant role in non-amyloid light chain deposition disease (LCDD), this condition is as yet poorly documented at the molecular level, since only three sequences have been reported to date. In a case of myeloma-associated LCDD, the patient's urine contained an unglycosylated kappa Bence Jones protein made up of dimers and monomers with an apparent molecular mass of 25,000 which was assigned to the V kappa I subgroup by N-terminal amino acid sequencing. The complete variable region sequence of the monoclonal kappa chain produced by the malignant plasma cells was amplified by polymerase chain reaction (PCR) using small amounts of material obtained by bone marrow aspiration. The sequence of three independently amplified cDNA clones derived from a normal-sized kappa messenger RNA was identical to that of the urinary kappa chain. The kappa mRNA had an overall normal structure made up of a V kappa I sequence rearranged to J kappa I. Several unusual features of the variable region (the first complete V kappa I sequence reported in LCDD) included three substitutions that introduced hydrophobic residues at spatially close positions. The strategy associating N-terminal sequence determination and cDNA cloning by PCR could help in accumulating new sequence data and improving our understanding of LCDD pathogenesis. FAU - Rocca, A AU - Rocca A AD - CNRS URA 1172, Laboratory of Molecular Immunology, Faculty of Sciences and University Hospital, Poitiers, France. FAU - Khamlichi, A A AU - Khamlichi AA FAU - Aucouturier, P AU - Aucouturier P FAU - Noel, L H AU - Noel LH FAU - Denoroy, L AU - Denoroy L FAU - Preud'homme, J L AU - Preud'homme JL FAU - Cogne, M AU - Cogne M LA - eng SI - GENBANK/X67322 SI - GENBANK/X71069 SI - GENBANK/X71070 SI - GENBANK/X71071 SI - GENBANK/X71675 SI - GENBANK/X71676 SI - GENBANK/X71677 SI - GENBANK/X71678 SI - GENBANK/X71679 SI - GENBANK/X71680 PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Clin Exp Immunol JT - Clinical and experimental immunology JID - 0057202 RN - 0 (Immunoglobulin G) RN - 0 (Immunoglobulin kappa-Chains) RN - 63231-63-0 (RNA) RN - 9006-99-9 (Bence Jones Protein) SB - IM MH - Amino Acid Sequence MH - Base Sequence MH - Bence Jones Protein/genetics MH - Bone Marrow MH - Electrophoresis, Polyacrylamide Gel MH - Humans MH - Immunoglobulin G/*genetics MH - Immunoglobulin kappa-Chains/*genetics MH - Molecular Sequence Data MH - Multiple Myeloma/genetics MH - Paraproteinemias/*genetics MH - Polymerase Chain Reaction MH - RNA/genetics PMC - PMC1554718 EDAT- 1993/03/01 00:00 MHDA- 1993/03/01 00:01 PMCR- 1994/03/01 CRDT- 1993/03/01 00:00 PHST- 1993/03/01 00:00 [pubmed] PHST- 1993/03/01 00:01 [medline] PHST- 1993/03/01 00:00 [entrez] PHST- 1994/03/01 00:00 [pmc-release] AID - 10.1111/j.1365-2249.1993.tb05932.x [doi] PST - ppublish SO - Clin Exp Immunol. 1993 Mar;91(3):506-9. doi: 10.1111/j.1365-2249.1993.tb05932.x.