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The mysterious immunoglobulin light chain
Developmental & Comparative Immunology, 2002The immunoglobulin light chain is mysterious from different points of view. In an evolutionary perspective there seems to be at least three major pathways but it is today impossible to say which of them is the most ancient one and which isotype belongs to which branch.
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Blood, 2020
Light chain deposition disease (LCDD) is a rare disorder characterized by glomerular and peritubular amorphous deposits of a monoclonal immunoglobulin (Ig) light chain (LC), leading to nodular glomerulosclerosis and nephrotic syndrome. We developed a transgenic model using site-directed insertion of the variable domain of a pathogenic human LC gene ...
Christophe Sirac +19 more
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Light chain deposition disease (LCDD) is a rare disorder characterized by glomerular and peritubular amorphous deposits of a monoclonal immunoglobulin (Ig) light chain (LC), leading to nodular glomerulosclerosis and nephrotic syndrome. We developed a transgenic model using site-directed insertion of the variable domain of a pathogenic human LC gene ...
Christophe Sirac +19 more
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Treatment of immunoglobulin light chain amyloidosis
Current Hematologic Malignancy Reports, 2009No therapy is uniformly effective in the management of immunoglobulin light chain amyloidosis (AL amyloidosis). Despite the common generalization, therapy is highly effective. Options available to patients with AL amyloidosis include high-dose therapy, but this is applicable to only about one fourth of patients.
Morie A, Gertz, Steven R, Zeldenrust
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Immunoglobulin light chains, glycosaminoglycans, and amyloid
Cellular and Molecular Life Sciences, 2000Immunoglobulin light chains are the precursor proteins for fibrils that are formed during primary amyloidosis and in amyloidosis associated with multiple myeloma. As found for the approximately 20 currently described forms of focal, localized, or systemic amyloidoses, light chain-related fibrils extracted from physiological deposits are invariably ...
F J, Stevens, R, Kisilevsky
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Whale immunoglobulins—I. Light chain types
Comparative Biochemistry and Physiology Part B: Comparative Biochemistry, 1972Abstract 1. 1. Immunoglobulins (7S) were purified from the plasmas of three species of whales, hump-backed, fin-backed and Sei. 2. 2. Light polypeptide chains were characterized and compared to human light chains. 3. 3. Tryptic peptide maps and C-terminal analyses indicated the presence of both κ and λ light chains buth λ predominating ...
J C, Travis, B G, Sanders
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Immunoglobulin Light Chain Genes
1987The immunoglobulin genes are built from variable, joining, and constant gene segments (V, J, C). The V and J+C gene segments are separate in germline DNA and are brought into close proximity in the immunoglobulin-producing lymphoid cells. Antibody diversity rests mainly on the existence of many different V and several different J gene segments, on the ...
H. G. Zachau +6 more
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Nonchemotherapy Treatment of Immunoglobulin Light Chain Amyloidosis
Acta Haematologica, 2020Immunoglobulin light chain amyloidosis (AL amyloidosis) is a rare, life-threatening disease characterized by the deposition of misfolded proteins in vital organs such as the heart, the lungs, the kidneys, the peripheral nervous system, and the gastrointestinal tract. This causes a direct toxic effect, eventually leading to organ failure. The underlying
Layla, Van Doren, Suzanne, Lentzsch
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Immunoglobulin light chain nephropathies.
Pathology annual, 1987Excessive monoclonal light chain production and excretion may result in a variety of renal diseases which may collectively or individually be referred to as light chain nephropathy. Kappa light chains are more likely to produce tubular dysfunction and nodular nonamyloidotic glomerulosclerosis, while lambda light chains are more likely to be involved in
B C, Sturgill, F L, Tucker, W K, Bolton
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Immunoglobulin Light Chain and Systemic Light-Chain Amyloidosis
2007Light-chain amyloidosis (AL) is characterized by the clonal expansion of plasma B cells that secrete large amounts of monoclonal immunoglobulin light chains. The free light chains circulate in serum and form amyloid fibrils on vital organs such as the kidney, heart, and liver causing organ failure and eventually death.
Marina Ramirez-Alvarado +5 more
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Dispersed human immunoglobulin κ light-chain genes
Nature, 1986The gene segments encoding the constant and variable regions of human immunoglobulin light chains of the kappa type (C kappa, V kappa) have been localized to chromosome 2. The distance between the C kappa and V kappa genes and the number of germline V kappa genes are unknown.
E, Lötscher +5 more
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