8 nots - 1 October 1 2008 Transmembrane and FACIT collagens...

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1 October 1, 2008 Lecture 8 Transmembrane and FACIT collagens Collagenous transmembrane proteins Collagen XVII belongs to the family of collagenous transmembrane proteins, which are a biologically versatile group with double functions of cell-surface receptors and matrix molecules. Type XVII collagen, the focus of the lecture, is the best studied of the group. Powell et al (2005) Collagen XVII/BP180: a collagenous transmembrane protein and component of the dermoepidermal anchoring complex. Clinical and Experimental Dermatology 30: 682-687. Most of the information below is verbatim from this review with minor modifications or as otherwise noted. Summary Collagen XVII or BP180 (initially identified as the 180-kDa bullous pemphigoid antigen and also referred to as BPAg2) is a collagenous transmembrane protein and a structural component of the dermoepidermal anchoring complex. Molecular studies reveal that it has a globular cytosolic amino-terminal domain and flexible-rod extracellular carboxy terminal domain. Cell biological analyses suggest that collagen XVII functions as a cell- matrix adhesion molecule through stabilization of the hemidesmosome complex. This concept is supported by investigations into human diseases of the dermoepidermal junction, in which collagen XVII is either genetically defective or absent (as in some forms of nonlethal junctional epidermolysis bullosa). Autoantibodies against collagen XVII are seen in bullous pemphigoids. In vivo and in vitro studies provide evidence for a pathogenic role of these autoantibodies, and suggest that the serum level and epitope specificity of these antibodies influences disease severity and phenotype. The review summarizes the structural and biological features of collagen XVII and its role in diseases of the basement membrane zone. pemphigoid http://www.answers.com/topic/pemphigoid?cat=health An autoimmune skin disorder resembling characterized by bloody blisters, especially on the trunk and limbs. Collagen XVII and hemidesmosomes Collagen XVII is a structural component of the dermoepidermal anchoring complex, projecting beneath hemidesmesomes (HD) mediating adhesion of epidermal keratinocytes and certain other epithelial cells to the underlying basement membranes. The HD multiprotein complex, determine cell-stromal coherence and provide cells with cues critical for their polarization and spatial organization and for tissue architecture. Although these structures appear relatively stable, their functional activity can be modulated to facilitate cell migration. Regulation of the adhesive interactions of HD with the underlying basement membrane is essential in various biological processes, such as during morphogenesis, differentiation or regeneration.
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2 The Structure of collagen XVII Collagen XVII is a homodimer consisting of three identical α 1(XVII) chains. It is a type II transmembrane protein with a cytosolic N-terminus and extracellular C-
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8 nots - 1 October 1 2008 Transmembrane and FACIT collagens...

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