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HGNC:17935 2p13 MIM.604862 Entrez:50489

Thursday 9 October 2008

The protein CD207 encoded by this gene is expressed only in Langerhans cells which are immature dendritic cells of the epidermis and mucosa.

CD207 is localized in the Birbeck granules, organelles present in the cytoplasm of Langerhans cells and consisting of superimposed and zippered membranes.

CD207 is a C-type lectin with mannose binding specificity, and it has been proposed that mannose binding by this protein leads to internalization of antigen into Birbeck granules and providing access to a nonclassical antigen-processing pathway. [RefSeq]

GeneRIF (January 2010)

- Transgenic langerin effectively mediates antigen presentation in vivo by targeting its receptor in appropriate mouse dendritic cell subsets of draining lymph nodes and spleen; peptide major histocompatibility I and II complexes persist for days.

- Immunohistochemical evaluation of langerin expression may have utility in substantiating a diagnosis of Langerhans cell histiocytosis and separating this disorder from other non-Langerhans cell histiocytic proliferations.

- The carbohydrate-recognition domain of human Langerin was crystallized followed by X-ray analyses to resolutions of 2.5A for apo-Langerin and to 1.6A and 2.1A for the complexes with mannose and maltose, respectively.

- Electron microscopy analysis demonstrated a colocalization of human papillomavirus 16 virus-like particles and langerin, which is expressed only by Langerhans cells

- Langerin prevents HIV-1 transmission by Langernhans cells.

- The Langerin-positive cells strictly colonized the epidermis and no cells were found in the dermis.

- CD1a and langerin have roles in antigen presentation to T cells

- Langerin is a potent langerhans cells-specific regulator of membrane superimposition and zippering, representing a key molecule to trace Langerhans cells.

- The effects of the amino acid changes on the activity of langerin were examined by expressing each of the polymorphic forms.

- Langerin is expressed by Langerhans cells.

- Expression of mutated Langerin in human fibroblasts induces tubular-like structures that are negative for Birbeck granules-specific antibodies and do not resemble the characteristic structural features of Birbeck granules.

Animal models

- Langerin transgenic mice have substantially reduced numbers of epidermal langerhans cells, demonstrating that TGFbeta1 acts directly on these cells.


- Lau SK, Chu PG, Weiss LM. Immunohistochemical Expression of Langerin in Langerhans Cell Histiocytosis and Non-Langerhans Cell Histiocytic Disorders. Am J Surg Pathol. 2008 Feb 14; PMID: 18277880

- de Witte L, Nabatov A, Geijtenbeek TB. Distinct roles for DC-SIGN(+)-dendritic cells and Langerhans cells in HIV-1 transmission. Trends Mol Med. 2007 Dec 3; PMID: 18055263

- de Witte, L.; Nabatov, A.; Pion, M.; Fluitsma, D.; de Jong, M. A. W. P.; de Gruijl, T.; Piguet, V.; van Kooyk, Y.; Geijtenbeek, T. B. H. : Langerin is a natural barrier to HIV-1 transmission by Langerhans cells. Nature Med. 13: 367-371, 2007. PubMed ID : 17334373

- Chikwava K, Jaffe R. Langerin (CD207) staining in normal pediatric tissues, reactive lymph nodes, and childhood histiocytic disorders. Pediatr Dev Pathol. 2004 Nov-Dec;7(6):607-14. PMID: 15630529

- Valladeau, J.; Clair-Moninot, V.; Dezutter-Dambuyant, C.; Pin, J.-J.; Kissenpfennig, A.; Mattei, M.-G.; Ait-Yahia, S.; Bates, E. E. M.; Malissen, B.; Koch, F.; Fossiez, F.; Romani, N.; Lebecque, S.; Saeland, S. : Identification of mouse langerin/CD207 in Langerhans cells and some dendritic cells of lymphoid tissues. J. Immun. 168: 782-792, 2002. PubMed ID : 11777972

- Valladeau, J.; Ravel, O.; Dezutter-Dambuyant, C.; Moore, K.; Kleijmeer, M.; Liu, Y.; Duvert-Frances, V.; Vincent, C.; Schmitt, D.; Davoust, J.; Caux, C.; Lebecque, S.; Saeland, S. : Langerin, a novel C-type lectin specific to Langerhans cells, is an endocytic receptor that induces the formation of Birbeck granules. Immunity 12: 71-81, 2000. PubMed ID : 10661407