Birbeck granules, also known as Birbeck bodies, are rod shaped [1] or "tennis-racket" cytoplasmic organelles with a central linear density and a striated appearance. First described in 1961 (where they were simply termed "characteristic granules"), [2] they are solely found in Langerhans cells. [1] Although part of normal Langerhans cell histology, they also provide a mechanism to differentiate Langerhans cell histiocytoses (which are a group of rare conditions collectively known as histiocytoses) from proliferative disorders caused by other cell lines.
The function of Birbeck granules is debated, but one theory is that they migrate to the periphery of the Langerhans cells and release their contents into the extracellular matrix. Another theory is that the Birbeck granule functions in receptor-mediated endocytosis, similar to clathrin-coated pits.
Birbeck granules were discovered by Michael Stanley Clive Birbeck (1925–2005), a British scientist and electron microscopist who worked at the Chester Beatty Cancer Research Institute or Institute of Cancer Research, London from 1950 until 1981. [4]
Langerhans cells (LC) are tissue-resident macrophages of the skin, and contain organelles called Birbeck granules. They are present in all layers of the epidermis and are most prominent in the stratum spinosum. They also occur in the papillary dermis, particularly around blood vessels, as well as in the mucosa of the mouth, foreskin, and vaginal epithelium. They can be found in other tissues, such as lymph nodes, particularly in association with the condition Langerhans cell histiocytosis (LCH).
Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells, abnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes.
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Erdheim–Chester disease (ECD) is a rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages. It was declared a histiocytic neoplasm by the World Health Organization in 2016. Onset typically is in middle age. The disease involves an infiltration of lipid-laden macrophages, multinucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones.
Langerin (CD207) is a type II transmembrane protein which is encoded by the CD207 gene in humans. It was discovered by scientists Sem Saeland and Jenny Valladeau as main part of Birbeck granules. Langerin is C-type lectin receptor on Langerhans cells (LCs) and in mice also on dermal interstitial CD103+ dendritic cells (DC) and on resident CD8+ DC in lymph nodes.
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