Protein Bence-Jones immunoglobulin

Bacteriorhodopsin, see Purple membrane protein Bence-Jones protein, see Immunoglobulin... 

Wochner, R.D. Strober, W. and Waldman, T.A. The role of the kidney in catabolism of Bence Jones proteins and immunoglobulin fragments. J. Fxper Med 126 207-221, 1967. 

Bence Jones proteins Light chain immunoglobulins found in the urine. 

J. W. Longworth, C. L. McLaughlin, and A. Solomon, Luminescence studies on Bence-Jones proteins and light chains of immunoglobulins and their subunits, Biochemistry 14, 2953-2959 (1976). 

Another example that I now recall of poor fit between shape of a binding site and that of ligand is the immunoglobulin, or Bence-Jones protein, that binds the hapten phosphorylcholine. The X-ray structure of the antibody has been worked out and it is evident that the binding area is much larger than that of the hapten, and hardly complementary in shape. 

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). 

Another crucial development was the finding that the BALB/c (Potter, 1972, 1977a) and later that the NZB strains of mice (Warner, 1975) when injected with paraffin oil develop a disease like multiple myeloma and also often excrete Bence Jones proteins. This not only provided an experimental model, but also permitted detailed comparison of mouse Bence Jones proteins and immunoglobulins with their human counterparts, an indispensable prerequisite for the study of antibody specificity. Relatively enormous quantities (kilograms in some instances) of Bence Jones proteins were obtainable from the urine of patients plasmapheresis yielded substantial quantities of myeloma proteins. Large amounts of the corresponding mouse proteins were also obtainable proteins from such neoplasms were in almost all instances monoclonal and homogeneous. 

In addition to their unique individual specificity, Bence Jones proteins from different individuals were shown by immunological methods to fall into two classes (Korngold and Lipari, 1956), now termed k and A all five classes of immunoglobulins may contain either k or k light chains. Within k light chains, certain positions, 152 (Kern) and 190 (Inv), respectively, may have two amino acids, the genes for which are Mendelian alleles (Fig. 4). 

The existence of such variation in the N-terminal half of the light chains while the C-terminal half remained constant was a new phenomenon in protein chemistry. As the number of sequences increased and as such data on mouse Bence Jones proteins and later on heavy chains of immunoglobulins were accumulated, it became clear that understanding the nature of the structural and genetic basis for the variable domains of both chains was crucial to understanding antibody specificity. 

Immunoglobulin fi agments in urine or serum in urine, usually Bence Jones protein in serum, occasionally other fragments, such as monomeric IgM or heavy chain fragments. 

Our best evidence of this is a 3-year follow-up of 402 patients in whom Bence Jones protein had been detected in our laboratory. Dr. Corbett obtained biopsy evidence of malignant immunocytoma in 400. The various forms such malignant immunocytomata can take are listed in Table 7, which also includes the benign varieties in which I personally have not yet found immunoglobulin fragments. 

II. Establishing the level and class of a paraprotein enables a better appreciation of that level, and possible associations. Immunoglobulin fragments (urine should be properly examined for Bence Jones protein IgM for 7S, etc.) and reductions of other immunoglobulins imply a serious prognosis. Follow-up is essential and can indicate the natural history or response and escape on treatment. 

Bence Jones proteins (BJP), members of the paraproteins often found in the blood and urine of patients with multiple myeloma consisting of free light chains of immunoglobulins. 

Immunoglobulins.—Crystallographic studies of immunoglobulins are under way in a number of laboratories. These include studies of complete immunoglobulin molecules, of Bence-Jones proteins, and of fragments. 

Edmundson et alP have also studied crystals of the A-type Bence-Jones protein (the light chain of the immunoglobulin) from the same myeloma patient. The protein crystallizes in orthorhombic form from deionized water and as a trigonal form from ammonium sulphate. The orthorhombic form of space group P2i2i2 contains the M-S-S-M dimer. As with the euglobulin the salt-free crystals dissolved in most heavy-atom solutions, but PtCl made the crystals less soluble after which they could be treated by dilute solutions of heavy-atom salts. The rotation function has demon-... 

Bence Jones proteins are L chains (monomers or dimers k or X) synthesized by patients with multiple myeloma or, infrequently, with Waldenstrom s disease. The L chains are present in the serum and urine. They are often easy to isolate from urine because of their relatively high concentration and the absence of other immunoglobulins. About 10-15% of patients with multiple myeloma produce only a Bence Jones protein, and about 40% synthesize a myeloma protein without significant amounts of a Bence Jones protein. When both types of protein are present in an individual the Bence Jones protein is generally identical in structure to the L chain of the serum myeloma protein it therefore reflects the synthesis of excess L chains, as compared to H chains, by the malignant clone of cells. 

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