Bence Jones protein1
Bence Jones protein
A crystal of Bence Jones protein.
Bence Jones protein
is a monoclonal globulin protein orimmunoglobulin light chain found in the urine, with a molecularweight of 22-24 kDa.
Detection of Bence Jones protein may besuggestive of multiple myeloma or Waldenström's macroglobulinemia.Bence Jones proteins are particularly diagnostic of multiple myelomain the context of end-organ manifestations such as renal failure, lytic(or "punched out") bone lesions, anemia, or large numbers of plasmacells in the bone marrow of patients. Bence Jones proteins are presentin 2/3 of multiple myeloma cases.
The proteins are immunoglobulin light chains (paraproteins) and are produced by neoplastic plasma cells. They canbe kappa (most of the time) or lambda.
The light chains can be immunoglobulin fragments or single homogeneousimmunoglobulins. They are found in urine due to the kidneys' decreased filtration capabilities due to renal failure,sometimes induced by hypercalcemia from the calcium released as the bones are destroyed or from the light chainsthemselves. The light chains have historically been detected by heating and now by electrophoresis of concentratedurine. More recently serum free light chain assays have been utilised in a number of published studies which haveindicated superiority over the urine tests, particularly for patients producing low levels of monoclonal free lightchains, as seen in nonsecretory multiple myeloma
and AL amyloidosis.
This is primarily because of the re-absorption of free light chains in the kidneys, creating a "threshold" of light chain production which must beexceeded before measurable quantities overflow into the urine. As such, urinalysis is a fickle witness to changingfree light chain production.
The Bence Jones protein was described by the English physician Henry Bence Jones in 1847 and published in1848.
The protein was later sequenced by Frank W. Putnam Sr. at the laboratory of Fred Sanger in Cambridge,who was the first to report the entire sequence.
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