Bence Jones proteins

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. 

Klafki, H. W., Pick, A. I., Pardowitz, I., Cole, T., Awni, L. A., Barnikol, H. U., Mayer, F., Kratzin, H. D., and Hilschmann, N. (1993). Reduction of disulfide bonds in an amyloidogenic Bence Jones protein leads to formation of amyloid-like fibrils in vitro. Biol. Chem. Hoppe Seyfer374, 1117-1122. 

Boc-E4 Boc-EAR lR-MCA BJP-B6 + MCA Autoantibodies Bence Jones proteins (BJPs) (monoclonal antibody light chains) isolated from the urine of multiple myeloma patients, were found to hydrolyse peptide methylcoumarin amide peptide-MCA substrates 1.5 x 101 3.3 X 1()-2 nr 5.8... 

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). 

Fig. 33. Stereo drawings of particular examples of types Via (a) and VIb (b) cis-proline turns, (a) Ribonuclease S 91-94 (Lys-Tyr-Pro-Asn) (b) Bence-Jones protein REI 6-9 (Gln-Ser-Pro-Ser).

Fig. 37. plot for the cis-proline (type VI) turns from Chou and Fasman (1977), plus the two examples in the Bence-Jones protein REI. Arrows point from position 2 to position 3 (the proline) for each example. The two conformational groups are labeled as Via and VIb. 

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

Specific gravity Gram stain Bence-Jones protein Paraguat test Porphobilinogen Myoglobin Pregnancy tset Fractional urinalysis 1.016-1.022... 

It cannot be overemphasized that the absence of a monoclonal band of protein in the serum of a patient suspected of having multiple myelomatosis does not rule out the diagnosis. Quite frequently, the suspected patient may be excreting all the paraprotein in the form of a Bence Jones protein into the urine, and hence the simultaneous examination of both the serum and the urine by electrophoresis usually jirovides a rapid and excellent means of diagnosis. 

On examination of the urine and serum of numerous patients with suspected paraproteinemia in both Jamaicans and Africans between 1962 and 1966, it was concluded that whenever a low total serum y-globulin level with a normal serum electrophoretic pattern were encountered in a suspected case of multiple myelomatosis, it was then essential to obtain also a specimen of urine from such a patient for further electrophoretic examination. Invariably simultaneous electrophoresis of such sera and urines proved to be diagnostic, even when the classical heat test for Bence Jones protein was negative. Consequently it was found that concurrent electrophoresis of scrum and urine was the first means of detecting multiple myelomatosis in no less than 20% of the patients, which were subsequently confirmed either by bone marrow biopsy or X-ray examination or both (M3). 

The commonest causes of death in the Jamaican patients with multiple myelomatosis were bronchopneumonia and other infectious complications, a finding which is compatible with the secondary antibody deficiency syndrome and impaired cellular immunity which occurs in patients with this disease. Bleeding manifestations and renal failure were not uncommon findings, and myeloma kidney was observed in 66% of the cases. Skeletal involvement was observed, but in many cases the typical lesions had to be searched for. Amyloidosis was present in as many as 21% of the patients (Tl) and this may be associated with the high number of patients in Jamaica that are known to excrete Bence Jones protein in myeloma (Mil). 

Ultracentrijugation. In those laboratories where an ultracentrifuge is available, the pattern obtained of the serum of a patient with Waldenstrom macroglobulinemia is very characteristic. Frequently more than 30% of the total serum proteins could be accounted for by the 19 S peak. Bence Jones protein is not an uncommon finding in patients with Waldenstrom macroglobulinemia and was detected in patients both in Nigeria and Jamacia with the disease. 

Isophorone produced kidney effects in male rats in the NTP (1986) study. Strasser (1988) found that isophorone caused protein droplet formation in the kidneys of male rats, suggesting that isophorone can induce protein nephropathy. Alden (1986) discussed the possibility that proteinuric humans and humans with low molecular weight protein nephropathy, such as people with multiple myeloma (Bence-Jones protein) or mononuclear cell leukemia (lysozyme), may be more susceptible to chemically-induced protein nephropathy. He concluded, however, that this syndrome is probably specific to the male rat. 

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. 

Figure 31-4 Schematic structure of one-fifth of an IgM molecule. From Putnam et al A (A) Covalent structure. (B) Schematic three-dimensional representation. (C) Ribbon diagram of an IgG molecule. From Cochran et al,64a (D) Folding patterns of one chain in a constant and a variable domain of a Bence-Jones protein. From Schiffer et al.66 Green arrows indicate hypervariable regions. (E) MolScript drawing of the common core structure of Ig-like domains. The lighter shaded strands (b, c, e, f) form the core common to all Ig-like domains, which is surrounded by structurally more varied additional strands (darker). The front sheet has up to five strands (a, f, c, e, c") and the back sheet up to four (a, b, e, d). Strand c" is very flexible and is not always a part of the (3 sheet. From Bork, Holm, and Sander.65 See also Fig. 2-16.

Appella, E., McIntyre, K.R., Perham, R.N. (1967). Lambda Bence Jones proteins of the mouse chemical and immunological characterization. J. Mol. Biol. 27, 391-394. 

Wu, T.T. Rabat, E.A. (1970). An analysis of the sequences of the variable regions of Bence Jones proteins and myeloma light chains and their implications for antibody complementarity. J. Exp. Med. 132,211-250. 

Monoclonal protein can be detected in serum, urine, or both in greater than 95% of patients with multiple myeloma (D16). Bone marrow plasma cells exceed 10%. Patients with advanced disease may excrete Bence-Jones proteins in urine. Both hypercalcemia and Bence-Jones proteinuria can contribute to renal failure (A6). 

Unless M protein concentrations exceed 60 g/L, patients with multiple myeloma do not develop hyperviscosity syndrome. Despite the presence of plasma cells in bone marrow exceeding 10% and the presence of M protein exceeding 25 g/L, nearly 15% of patients with multiple myeloma are asymptomatic. However, asymptomatic patients presenting with IgA myeloma protein and M protein concentrations exceeding 30 g/L and Bence Jones-protein excretion in excess of 50 mg/day in presence of a lytic bone lesion can progress to multiple myeloma earlier than other, asymptomatic patients (Wl). 

The distinction between essential monoclonal gammopathy and asymptomatic multiple myeloma can be difficult in subjects whose M protein and Bence-Jones protein ranges and percentage of plasma cells in bone marrow overlap. 

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). 

Wallis and Engle (W2) have reported cases among adults ( Milkman syndrome ), the condition being eventually attributed to the toxicity of heavy metals or of Bence Jones proteins (E3). According to Dent and Harris (Dll) the condition is transmitted as a recessive gene. 

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