Human epidermal Langerhans cells express the tight junction protein claudin-1 and are present in human genetic claudin-1 deficiency (NISCH syndrome).

Details

Serval ID
serval:BIB_BF0335A1C522
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Human epidermal Langerhans cells express the tight junction protein claudin-1 and are present in human genetic claudin-1 deficiency (NISCH syndrome).
Journal
Experimental Dermatology
Author(s)
Zimmerli S.C., Kerl K., Hadj-Rabia S., Hohl D., Hauser C.
ISSN
1600-0625[electronic]
Publication state
Published
Issued date
2008
Volume
17
Number
1
Pages
20-23
Language
english
Abstract
Claudin-1 (CLDN1) is a structural tight junction (TJ) protein and is expressed in differentiating keratinocytes and Langerhans cells in the epidermis. Our objective was to identify immunoreactive CLDN1 in human epidermal Langerhans cells and to examine the pattern of epidermal Langerhans cells in genetic human CLDN1 deficiency [neonatal ichthyosis, sclerosing cholangitis (NISCH) syndrome]. Epidermal cells from healthy human skin labelled with CLDN1-specific antibodies were analysed by confocal laser immunofluorescence microscopy and flow cytometry. Skin biopsy sections of two patients with NISCH syndrome were stained with an antibody to CD1a expressed on epidermal Langerhans cells. Epidermal Langerhans cells and a subpopulation of keratinocytes from healthy skin were positive for CLDN1. The gross number and distribution of epidermal Langerhans cells of two patients with molecularly confirmed NISCH syndrome, however, was not grossly altered. Therefore, CLDN1 is unlikely to play a critical role in migration of Langerhans cells (or their precursors) to the epidermis or their positioning within the epidermis. Our findings do not exclude a role of this TJ molecule once Langerhans cells have left the epidermis for draining lymph nodes.
Keywords
Biopsy, Cell Movement, Cholangitis, Sclerosing/metabolism, Cholangitis, Sclerosing/pathology, Humans, Ichthyosis/metabolism, Ichthyosis/pathology, Keratinocytes/metabolism, Keratinocytes/pathology, Langerhans Cells/metabolism, Langerhans Cells/pathology, Membrane Proteins/metabolism, Skin/metabolism, Skin/pathology, Syndrome, Tight Junctions/metabolism
Pubmed
Web of science
Create date
02/10/2009 17:26
Last modification date
20/08/2019 15:33
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