PMID- 9409653 OWN - NLM STAT- MEDLINE DCOM- 19980108 LR - 20190512 IS - 0009-9104 (Print) IS - 1365-2249 (Electronic) IS - 0009-9104 (Linking) VI - 110 IP - 3 DP - 1997 Dec TI - Amino-terminal identity of co-existent amyloid and non-amyloid immunoglobulin kappa light chain deposits. A human disease to study alterations of protein conformation. PG - 472-8 AB - Tissue deposition of monoclonal immunoglobulin light chains is a serious complication in some patients with B cell proliferative disorders. The deposits are typically fibrillar and Congophilic in amyloid (AL) and non-fibrillar and Congophobic in light chain deposition disease (LCDD), and rarely coexist in the same patient. From post-mortem tissue of an individual with fibrillar and non-fibrillar kappa light chain deposits in different sites, we separately extracted and analysed biochemically and immunochemically the non-amyloid deposits from isolated glomeruli, the amyloid from isolated renal arteries and the amyloid from myocardium in which the only deposits were amyloid restricted to mural arteries. Western blotting analysis of both the extracted amyloid and the non-amyloid deposits demonstrated 25-kD bands immunoreactive with anti-kappa antibody, and the identity of the N-terminal amino acid sequences that belong to the variable region kappaIV light chain subgroup. This is the first human disease in which antigenically similar but morphologically different deposits have been separately biochemically analysed. We propose that combined LCDD and AL is an ideal human disease to study the relationships and the factors that influence the conversion of non-amyloidogenic to amyloidogenic conformations. FAU - Kaplan, B AU - Kaplan B AD - Department of Pathology, New York University Medical Centre, NY 10016, USA. FAU - Vidal, R AU - Vidal R FAU - Kumar, A AU - Kumar A FAU - Ghiso, J AU - Ghiso J FAU - Frangione, B AU - Frangione B FAU - Gallo, G AU - Gallo G LA - eng GR - AG0805/AG/NIA NIH HHS/United States GR - AR 02594/AR/NIAMS NIH HHS/United States PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, P.H.S. PL - England TA - Clin Exp Immunol JT - Clinical and experimental immunology JID - 0057202 RN - 0 (Amyloid) RN - 0 (Immunoglobulin kappa-Chains) SB - IM MH - Aged MH - Amino Acid Sequence MH - Amyloid/*chemistry MH - Amyloidosis/*metabolism MH - Humans MH - Hypergammaglobulinemia/*metabolism MH - Immunoglobulin kappa-Chains/*chemistry MH - Immunohistochemistry MH - Male MH - Molecular Sequence Data MH - Protein Conformation PMC - PMC1904831 EDAT- 1997/12/31 00:00 MHDA- 1997/12/31 00:01 PMCR- 1998/12/01 CRDT- 1997/12/31 00:00 PHST- 1997/12/31 00:00 [pubmed] PHST- 1997/12/31 00:01 [medline] PHST- 1997/12/31 00:00 [entrez] PHST- 1998/12/01 00:00 [pmc-release] AID - 10.1046/j.1365-2249.1997.4421454.x [doi] PST - ppublish SO - Clin Exp Immunol. 1997 Dec;110(3):472-8. doi: 10.1046/j.1365-2249.1997.4421454.x.