Abstract
Bone marrow cells from 3 6 patients with AL amyloid and 10 with either light chain or light and heavy chain deposition disease were incubated with radioactive amino acids culture and the Ig-related synthetic products analyzed. All synthesized excess free monoclonal L-chains corresponding to the class found in the tissue deposits. 12 of 32 AL patients had intracellular and/or secreted L-chain fragments. The cells from 5 of 10 deposition disease patients contained L or H fragments or both. H-chain fragments were seen only in the patients with H-chain tissue deposition. There was no evidence for proteolysis of normal sized chains as the mechanism of production of the fragments. Immunofluorescent analysis of tissue P-component demonstrated its presence only in Congo Red positive fibrillar deposits, even in patients whose tissues contained both types of Ig deposition.
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© 1991 Springer Science+Business Media Dordrecht
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Buxbaum, J., Caron, D., Gallo, G. (1991). AL Amyloid, L-Chain And L & H-Chain Deposition Diseases: Comparison of Ig Synthesis and Tissue Deposition.. In: Natvig, J.B., et al. Amyloid and Amyloidosis 1990. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-3284-8_49
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DOI: https://doi.org/10.1007/978-94-011-3284-8_49
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