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Gene Review

Col17a1  -  collagen, type XVII, alpha 1

Mus musculus

Synonyms: 180 kDa bullous pemphigoid antigen 2, BP180, BPAg2, Bp180, Bpag, ...
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Disease relevance of Col17a1


High impact information on Col17a1

  • Only NE degrades BP180 and produces dermal-epidermal separation in vivo and in culture [5].
  • Bullous pemphigoid (BP) is a blistering skin disease associated with an IgG autoimmune response directed against the ectodomain of the hemidesmosomal protein, BP180 [6].
  • Immunofluorescence microscopy of bovine skin revealed the basement membrane zone of skin to be stained clearly with mAb 1337, whereas the lateral surfaces of basal cells, which were decorated by typical antibodies against BP180, were not [7].
  • Therefore, the polypeptide was identified as an extracellular fragment of BP180. mAb 1337 immunoprecipitated the 120-kDa fragment from the medium, but not the 180-kDa molecule of BP180 extracted from cultured cells, indicating that the antibody specifically recognizes the fragment [7].
  • Restoration of full-length BP180 protein expression was associated with adhesion parameter normalization of primary JEB keratinocytes in vitro [4].

Chemical compound and disease context of Col17a1


Biological context of Col17a1

  • Bpag-2 mapped to the distal end of mouse chromosome 19 in a region of homology to human chromosome 10q [9].
  • The canine BPAg2 sequence was confirmed by a rabbit antibody raised against a 18-amino acid peptide deduced from the canine NC16A nucleotide sequence [10].
  • BP180 gene-transduced tissue demonstrated restoration of BP180 gene expression at the dermal-epidermal junction in vivo while untransduced regenerated JEB skin entirely lacked BP180 expression [4].
  • The C-terminal extracellular domain of BP180 contains 15 domains composed of Gly-X-Y tandem repeats, which are predicted to form collagen-like triple helices [8].

Anatomical context of Col17a1

  • Autoantibodies in BP react with BP180 and BP230, two major components of the hemidesmosome, a cell structure involved in dermal-epidermal adhesion [11].
  • Key immunohistological features of BP include dermo-epidermal junction (DEJ) separation, an inflammatory cell infiltrate in the upper dermis, and autoantibodies directed against two emidesmosomal antigens, BP230 and BP180 [12].
  • T cells from BP patients show a specific proliferative response to recombinant BP180 NC16A [13].
  • Breaking B-cell tolerance to BPAg2 sets the first step in dissecting the disease's induction phase [14].

Associations of Col17a1 with chemical compounds

  • Trehalose significantly prolonged the storage time of FL-BP180 expressed in Sf21 insect cells, thus permitting the routine use of the IF assay in clinics for the detection of serum antibodies [2].

Analytical, diagnostic and therapeutic context of Col17a1

  • We have recently developed a simple, highly specific and sensitive immunofluorescence (IF) assay for the detection of circulating antibodies against BP180 [2].
  • Our results suggest that indirect IF of 1 M NaCl-split skin and immunoblotting of a fusion protein representing the BP180 NC16a domain are more sensitive techniques for the diagnosis of HG than conventional and complement IF or immunoblotting of crude epidermal extracts [15].
  • Molecular cloning of canine bullous pemphigoid antigen 2 cDNA and immunomapping of NC16A domain by canine bullous pemphigoid autoantibodies [10].
  • Rotary shadow electron microscopy of affinity-purified 120-kDa fragments demonstrated that they have the unique molecular shape consisting of a central rod and a flexible tail, without the globular head that is present in the BP180 molecule [7].


  1. Macrophages, but not T and B lymphocytes, are critical for subepidermal blister formation in experimental bullous pemphigoid: macrophage-mediated neutrophil infiltration depends on mast cell activation. Chen, R., Fairley, J.A., Zhao, M.L., Giudice, G.J., Zillikens, D., Diaz, L.A., Liu, Z. J. Immunol. (2002) [Pubmed]
  2. Trehalose conserves expression of bullous pemphigoid antigen 180 during desiccation and freezing. Schmidt, E., Kromminga, A., Kürschner, M., Zimmermann, H., Katsen, A.D., Bröcker, E.B., Zillikens, D., Zimmermann, U., Sukhorukov, V.L. J. Immunol. Methods (2003) [Pubmed]
  3. Elevated expression and release of tissue-type, but not urokinase-type, plasminogen activator after binding of autoantibodies to bullous pemphigoid antigen 180 in cultured human keratinocytes. Schmidt, E., Wehr, B., Tabengwa, E.M., Reimer, S., Bröcker, E.B., Zillikens, D. Clin. Exp. Immunol. (2004) [Pubmed]
  4. BP180 gene delivery in junctional epidermolysis bullosa. Seitz, C.S., Giudice, G.J., Balding, S.D., Marinkovich, M.P., Khavari, P.A. Gene Ther. (1999) [Pubmed]
  5. The serpin alpha1-proteinase inhibitor is a critical substrate for gelatinase B/MMP-9 in vivo. Liu, Z., Zhou, X., Shapiro, S.D., Shipley, J.M., Twining, S.S., Diaz, L.A., Senior, R.M., Werb, Z. Cell (2000) [Pubmed]
  6. The role of complement in experimental bullous pemphigoid. Liu, Z., Giudice, G.J., Swartz, S.J., Fairley, J.A., Till, G.O., Troy, J.L., Diaz, L.A. J. Clin. Invest. (1995) [Pubmed]
  7. Cleavage of BP180, a 180-kDa bullous pemphigoid antigen, yields a 120-kDa collagenous extracellular polypeptide. Hirako, Y., Usukura, J., Uematsu, J., Hashimoto, T., Kitajima, Y., Owaribe, K. J. Biol. Chem. (1998) [Pubmed]
  8. A recombinant form of the human BP180 ectodomain forms a collagen-like homotrimeric complex. Balding, S.D., Diaz, L.A., Giudice, G.J. Biochemistry (1997) [Pubmed]
  9. Chromosomal localization of mouse bullous pemphigoid antigens. BPAG1 and BPAG2: identification of a new region of homology between mouse and human chromosomes. Copeland, N.G., Gilbert, D.J., Li, K., Sawamura, D., Giudice, G.J., Chu, M.L., Jenkins, N.A., Uitto, J. Genomics (1993) [Pubmed]
  10. Molecular cloning of canine bullous pemphigoid antigen 2 cDNA and immunomapping of NC16A domain by canine bullous pemphigoid autoantibodies. Xu, L., O'Toole, E.A., Olivry, T., Hernandez, C., Peng, J., Chen, M., Chan, L.S. Biochim. Biophys. Acta (2000) [Pubmed]
  11. The desmosome and hemidesmosome in cutaneous autoimmunity. Lin, M.S., Mascaró, J.M., Liu, Z., España, A., Diaz, L.A. Clin. Exp. Immunol. (1997) [Pubmed]
  12. Immunopathology of bullous pemphigoid, an autoimmune and inflammatory skin blistering disease. Liu, Z. The Keio journal of medicine. (2003) [Pubmed]
  13. Bullous pemphigoid: end of the century overview. Liu, Z., Diaz, L.A. J. Dermatol. (2001) [Pubmed]
  14. Characterization of BALB/c mice B lymphocyte autoimmune responses to skin basement membrane component type XVII collagen, the target antigen of autoimmune skin disease bullous pemphigoid. Xu, L., Robinson, N., Miller, S.D., Chan, L.S. Immunol. Lett. (2001) [Pubmed]
  15. Analysis of antigens recognized by autoantibodies in herpes gestationis. Usefulness of immunoblotting using a fusion protein representing an extracellular domain of the 180 kD bullous pemphigoid antigen. Murakami, H., Amagai, M., Higashiyama, M., Hashimoto, K., Chorzelski, T.P., Bhogal, B.S., Jenkins, R.E., Black, M.M., Zillikens, D., Nishikawa, T., Hashimoto, T. J. Dermatol. Sci. (1996) [Pubmed]
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