Light chain amyloidosis

Several pathological self-polymerizing systems have been biophysi-cally characterized sufficiently to permit identification of protein or peptide species that could serve as molecular targets in a structure-activity relationship. These include transthyretin (TTR) [73-76], serum amyloid A protein (SAA) [77], microtubule-associated protein tau [78-80], amylin or islet amyloid polypeptide (IAPP) [81,82], IgG light chain amyloidosis (AL) [83-85], polyglutamine diseases [9,86], a-synuclein [47,48] and the Alzheimer s (3 peptide [87-96]. A variety of A(3 peptide assay systems have been established at Parke-Davis to search for inhibitors of fibril formation that could be therapeutically useful [97]. 

There have been cases of acute tumor lysis syndrome in patients with melanoma (322) and light-chain amyloidosis (323). The authors reviewed the incidence of acute tumor lysis syndrome in these diseases, which are less typically associated with it. 

Light chain variable domain from light chain amyloidosis (SAM) Apl-42... 

Buxbaum, J.N. Chuba, J.V. Heilman, G.C. Solomon, A. Gallo, G.R. Monoclonal immunoglobulin deposition disease light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis. Clinical... 

Immunoglobulin light-chain, amyloidosis, myeloma-associated amyloidosis, monoclonal plasma cell dyscrasias... 

Helms LR, Wetzel R. Specificity of abnormal assembly in immunoglobulin light chain deposition disease and amyloidosis. J Mol Biol 1996 ... 

In this condition monoclonal immunoglobulins are produced due to a clonal disorder of either plasma cells or lymphocytes. Light chains produced are more frequently of the A than the k type (A7, S17). The monoclonal immunoglobulins produced could be either IgG, IgA, IgM, or IgD. The deposition of a fibrillar protein, most often k light chains, in many tissues is referred to as amyloidosis. The deposition of A. light chains alone is referred to as primary amyloidosis. 

Solomon, A., Frangione, B., and Franklin, E. C., Bence Jones proteins and light chains of immunoglobulins. Preferential association of the V lambda VI subgroup of human fight chains with amyloidosis AL (lambda). J. Clin. Invest. 70, 453-460 (1982). 

Cutaneous amyloidosis localized amyloidosis of the skin Lambda immunoglobulin light chains of variable subgroup I Systemic... 

Ganeval, D. Noel, L.H. Preud home, J.L. Droz, D. Grunfeld, J.P. Light-chain deposition disease its relation with AL-type amyloidosis. Kidney Int. 1984, 2d (1), 1-9. 

Buxbaum, J. Mechanisms of disease monoclonal immunoglobulin deposition. Amyloidosis, light chain deposition disease, and light and heavy chain deposition disease. Hematol./Oncol. Clin. North Am. 1992, 6 (2), 323-346. 

Bencejones (free light chains) only 20 56 100 Often renal failure bone lesions amyloidosis poor prognosis... 

AL amyloidosis is the most common form of these disorders. Bone marrow plasmacytosis and excess plasma cell production of antigenicaHy identical monoclonal light chains are common to primary amyloidosis and multiple myeloma. Thus a clear distinction between these two conditions is not possible chemically. They appear to differ only in the presence or absence of osteolytic lesions. AL deposits may occur in the tongue, heart, lymph nodes, spleen, joints, peripheral nerves, and skin. 

Amyloidosis is a term encompassing many diseases that share a common feature— the extracellular deposition of pathologic insoluble fibrillar proteins called amyloid in organs and tissues. In Amy Lloyd s disease, amyloidosis/AL, the amyloid is derived from immunoglobulin light chains (AL = amyloidosis, light-chain related). 

In AL amyloidosis, amyloid is formed from degradation products of the X or k light chains that deposit most frequently in the extracellular matrix of the kidney and the heart but also may deposit in the tongue. In other types of amyloidosis, the amyloid arises from other proteins and deposits in a characteristic organ. For example, the amyloid associated with chronic inflammatory conditions, such as tuberculosis or rheumatoid arthritis, is derived from an acute phase serum protein called serum amyloid Athat is produced by the liver in response to inflammation. It deposits most frequently in the kidney, and cardiac involvement is rare. 

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