Bence Jones protein
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Bence Jones proteins are proteins that are excreted in the urine in certain diseases (known as Bence Jones proteinuria ). Biochemically, they are immunoglobulin light chain proteins that are free ( i.e. not bound in intact immunoglobulins). As a rule, the underlying cause is a disease in which large quantities of light chain proteins are formed. Because these proteins have a relatively small molecular weight, they can pass the glomerular filter and are then reabsorbed and broken down by the cells of the proximal tubule . With a sufficiently high concentration or a disturbance of reabsorption in the proximal tubule, they can therefore appear in the urine and be detected.
The underlying disease is usually multiple myeloma , a malignant disease of plasma cells . Since this is a monoclonal disease, the free light chain proteins produced by these plasma cells are all of the same type - either " kappa " or " lambda ".
Light chain proteins can be deposited in various organs in the form of semi-crystalline structures, so-called amyloids . A primarily affected organ is the kidney, which leads to a functional disorder ( renal insufficiency ). This can progress to complete functional failure of the kidneys (chronic kidney failure ).
Bence Jones proteins can be detected in the urine by a number of tests, for example SDS-PAGE , immunofixation , immunoelectrophoresis or the free light chain determination .
history
The protein is named after Henry Bence Jones , who described it in 1848. The hematologist Robert A. Kyle assumes that this protein was described by Johann Florian Heller as early as 1846 . According to Kyle, Bence Jones is more likely to have made a connection between the occurrence of the protein and the cancer later known as multiple myeloma .
It was not until 1955 that Putnam and Hardy showed that the protein is not a metabolic breakdown product, but a synthesis product of cells.
literature
wikilite.com English language site with information on the discovery of the Bence Jones protein and monoclonal gammopathies in general.
Individual evidence
- ↑ a b C. Hutchison et al., The pathogenesis and diagnosis of acute kidney injury in multiple myeloma. Nature Reviews Nephrology . 2011 Nov 1; 8 (1): 43-51, PMID 22045243 .
- ^ Henry Bence Jones: On a new substance occurring in the urine of a patient with "mollities ossium." In: Phil Trans Royal Soc London. 1848, pp. 55-62. ( Full text )
- ↑ Information page about the free immunoglobulin light chains English-language page with information about the discovery of the Bence Jones protein.
- ^ RA Kyle: Henry Bence Jones - physician, chemist, scientist and biographer: a man for all seasons. In: British Journal of Hematology . Volume 115, Number 1, October 2001, pp. 13-18, PMID 11722404 .
- ^ FW Putnam, S. Hardy: Proteins in multiple myeloma. III. Origin of Bence-Jones protein. In: The Journal of biological chemistry . Volume 212, Number 1, January 1955, pp. 361-369, PMID 13233238 .