PMID: 2118650Sep 1, 1990Paper

Immunoglobulin heavy-chain-associated amyloidosis

Proceedings of the National Academy of Sciences of the United States of America
M EulitzA Solomon

Abstract

Immunoglobulin- or multiple myeloma-associated amyloidosis has been distinguished by the tissue deposition of Congophilic, fibrillar protein consisting of light chains or light-chain fragments (AL amyloidosis). We now report the isolation and characterization of another form of immunoglobulin-associated amyloid obtained from a patient who had extensive systemic amyloidosis and in whom the amyloid deposits consisted not of light chains but rather of an unusual form of heavy chain. This component, isolated from splenic amyloid extracts, represented an internally deleted IgG1 heavy chain as evidenced by immunochemical, electrophoretic, and amino acid sequence analyses. A comparable immunoglobulin-related monoclonal protein, consisting only of IgG heavy chains, was present in the patient's urine. Based on serologic reactivity with a battery of anti-immunoglobulin antisera, these two immunoglobulin-related components were antigenically identical; however, when compared to normal IgG, both were deficient in Fc-associated gamma-chain determinants. The structural abnormality of the amyloid gamma-chain protein was further evidenced by SDS/PAGE and immuno-blotting analyses: An unusually low molecular mass of approximately 22 kDa was foun...Continue Reading

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Citations

Feb 1, 1994·American Journal of Hematology·A SolomonC Murphy
Jul 16, 2005·Virchows Archiv : an International Journal of Pathology·Batia KaplanMordechai R Kramer
Dec 27, 2007·Clinical & Translational Oncology : Official Publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico·M J Molina-GarridoA Carrato
Mar 1, 1993·Molecular Immunology·R C WilliamsA Solomon
May 1, 1994·International Journal of Immunopharmacology·J L Preud'hommeM Cogné
Jul 1, 1995·Critical Reviews in Oncology/hematology·E Pascali
Dec 20, 2003·Blood Reviews·Morie A GertzAngela Dispenzieri
Nov 4, 1993·The New England Journal of Medicine·P AucouturierJ L Preud'homme
Nov 4, 1993·The New England Journal of Medicine·A Solomon, D T Weiss
Oct 28, 2004·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Lea NovakPaul W Sanders
Nov 8, 2011·American Journal of Physiology. Heart and Circulatory Physiology·Jian GuanRonglih Liao
Sep 17, 2005·American Journal of Respiratory and Critical Care Medicine·Andrew J SloaneJenny L Harry
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Jul 14, 2005·Amyloid : the International Journal of Experimental and Clinical Investigation : the Official Journal of the International Society of Amyloidosis·Ryota IkeeSoichiro Miura
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