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Treatment of immunoglobulin light chain amyloidosis

Current Hematologic Malignancy Reports, 2009
No 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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Light chain immunoglobulins as substrates for collagenase

Immunochemistry, 1975
Abstract The proteolytic action of collagenase on immunoglobulin light chains has been studied by mean of quantitative determination of N-terminal amino acid residues. L (λ) and L (κ) appear to be susceptible to the enzymatic action and this characteristics is a highly specific and general one.
M A, Coletti-Previero   +2 more
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Light chains of immunoglobulins in human secretions

Clinica Chimica Acta, 1994
Immunoglobulin kappa light chains are predominant in normal human serum and a kappa/lambda ratio of 1.7 to 2 has been reported. However, little is known of the partition of light chains in secretions. The levels of IgA, kappa and lambda were assayed by nephelometry in a series of secretions and in normal human serum.
C M, Molé   +4 more
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A Possible Precursor of Immunoglobulin Light Chains

Nature New Biology, 1972
IMMUNOGLOBULIN light chains are synthesized in heterologous cell-free systems from reticulocytes1 and Krebs II ascites cells2 and in reconstituted homologous systems from lymph nodes3,4 and myeloma tumours5,6. Although the synthesis appeared to be essentially faithful, absolute identity with authentic light chains was not demonstrated and some small ...
C, Milstein   +3 more
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Immunoglobulin light chain toxicity in a mouse model of monoclonal immunoglobulin light-chain deposition disease

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 ...
Bender, Sébastien   +19 more
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The mysterious immunoglobulin light chain

Developmental & Comparative Immunology, 2002
The 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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The immunoglobulin light chain in poikilothermic vertebrates

Immunological Reviews, 1998
Summary: The immunoglobulin light chains are classified as κ or δ in mammals and birds (homoeothermic vertebrates), but the traditional criteria for this classification are not applicable to the light chains found in poikilothermic vertebrates. Still it is possible to find some relationships between Ig light chain sequences in these animals and in ...
Lars Pilström   +5 more
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Immunoglobulin Light Chains and the Kidney: An Overview

Ultrastructural Pathology, 1994
Monoclonal immunoglobulins and free immunoglobulin light chains are produced by plasma cells as a result of their clonal expansion in plasma cell dyscrasia. These proteins are pivotal in the development of pathologic and clinical symptoms of plasma cell dyscrasia and renal manifestations are frequently the presenting and leading features of this ...
M M, Picken, S, Shen
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Free Light Chains of Immunoglobulins in Amyloidosis

Acta Medica Scandinavica, 1979
ABSTRACT Monomeric (M) and dimeric (D) forms of free light chains in serum have been measured by a sensitive radioimmunoassay in eight patients with amyloidosis without monoclonal proteins. Significantly elevated concentrations of D lambda chains were demonstrated in two of four patients with localized amyloidosis.
J, Sølling, K, Sølling
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Immunoglobulin light chains, glycosaminoglycans, and amyloid

Cellular and Molecular Life Sciences, 2000
Immunoglobulin 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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