Bence Jones protein
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 .
