Bence-Jones-Protein

Bence Jones proteins are proteins, which appear at the same Bence Jones proteinuria in the urine. These are low molecular weight, nephrotoxic ( poisonous to the kidneys ) paraproteins in the urine, consisting of two units of light chains of immunoglobulins (antibodies). It produces these proteins by degenerate plasma cells. These malignant cells occur in the so-called multiple myeloma in the bone marrow. In this condition it is in most cases (60-70 %) of a Bence-Jones proteinuria.

Bence Jones proteins can accumulate on various organs, which with the progression of the disease whose structure and functionality is disturbed in the body. Be mentioned is particularly the kidney, where the Bence Jones proteins in the glomerular capillaries deposit ( amyloidosis ) or in the distal renal tubules lead to functional impairment ( adult Fanconi syndrome) or fail ( precipitate ) and they can move. The one and the other can lead to progressive ( progressive ) kidney failure.

Bence Jones proteins in the urine may be detected by a number of tests, for example, SDS-PAGE, immunofixation or immunoelectrophoresis.

History

The protein is named after Henry Bence Jones, who described it in 1848. The hematologist Robert A. Kyle assumes that this protein has already been described in 1846 by Johann Florian Heller. Bence Jones stands by Kyle rather the merit of having a relationship between the occurrence of protein and cancer later than multiple myeloma designated manufactured.

Only Putnam and Hardy showed in 1955 that it is the protein is not a degradation product of metabolism, but a synthesis product of cells.