non fibrillar collagens


Summary: A family of structurally-related short-chain collagens that do not form large fibril bundles.

Top Publications

  1. Dowling J, Yang Y, Wollmann R, Reichardt L, Fuchs E. Developmental expression of BPAG1-n: insights into the spastic ataxia and gross neurologic degeneration in dystonia musculorum mice. Dev Biol. 1997;187:131-42 pubmed
    ..Collectively, our findings suggest a mechanism for the BPAG1 null phenotype and indicate that different neurons respond differently to the absence of BPAG1-n, a cytoskeletal linker protein. ..
  2. Verraes S, Hornebeck W, Polette M, Borradori L, Bernard P. Respective contribution of neutrophil elastase and matrix metalloproteinase 9 in the degradation of BP180 (type XVII collagen) in human bullous pemphigoid. J Invest Dermatol. 2001;117:1091-6 pubmed
    ..Our results confirm the importance of neutrophil elastase but not matrix metalloproteinase 9 in the direct cleavage of BP180 autoantigen and subepidermal blister formation in human bullous pemphigoid. ..
  3. Yamamoto K, Inoue N, Masuda R, Fujimori A, Saito T, Imajoh Ohmi S, et al. Cloning of hamster type XVII collagen cDNA, and pathogenesis of anti-type XVII collagen antibody and complement in hamster bullous pemphigoid. J Invest Dermatol. 2002;118:485-92 pubmed
    ..Under these conditions, IgG but not C3 was deposited on the basement membrane. These results strongly suggest that antigen-antibody complexes and complement initiate dermal-epidermal junction separation. ..
  4. Stanley J, Tanaka T, Mueller S, Klaus Kovtun V, Roop D. Isolation of complementary DNA for bullous pemphigoid antigen by use of patients' autoantibodies. J Clin Invest. 1988;82:1864-70 pubmed
    ..This BP cDNA clone will be valuable for understanding the protein structure, expression, and gene organization of BP antigen. ..
  5. Laffitte E, Skaria M, Jaunin F, Tamm K, Saurat J, Favre B, et al. Autoantibodies to the extracellular and intracellular domain of bullous pemphigoid 180, the putative key autoantigen in bullous pemphigoid, belong predominantly to the IgG1 and IgG4 subclasses. Br J Dermatol. 2001;144:760-8 pubmed
    ..These findings provide new insights relevant for our understanding of the immune response to BP180, the putative key autoantigen in BP. ..
  6. Brown A, Bernier G, Mathieu M, Rossant J, Kothary R. The mouse dystonia musculorum gene is a neural isoform of bullous pemphigoid antigen 1. Nat Genet. 1995;10:301-6 pubmed
    ..We also demonstrate abnormal dystonin transcripts in a second dt mutant, dt24J. We conclude that mutations in the dystonin gene are the primary genetic lesion in dt mice...
  7. Lin M, Fu C, Giudice G, Olague Marchan M, Lazaro A, Stastny P, et al. Epitopes targeted by bullous pemphigoid T lymphocytes and autoantibodies map to the same sites on the bullous pemphigoid 180 ectodomain. J Invest Dermatol. 2000;115:955-61 pubmed
    ..This new information will aid in defining the key steps involved in the development of the autoimmune response in bullous pemphigoid. ..
  8. Eyer J, Cleveland D, Wong P, Peterson A. Pathogenesis of two axonopathies does not require axonal neurofilaments. Nature. 1998;391:584-7 pubmed
    ..By specifically excluding a necessary role for axonal neurofilaments, our observations redefine the components of the pathogenic pathway leading to axon disruption in these two degenerative diseases...
  9. Nelson K, Zhao M, Schroeder P, Li N, Wetsel R, Diaz L, et al. Role of different pathways of the complement cascade in experimental bullous pemphigoid. J Clin Invest. 2006;116:2892-900 pubmed
    ..These findings provide the first direct evidence to our knowledge that complement activation via the classical and alternative pathways is crucial in subepidermal blister formation in experimental BP. ..

More Information


  1. Brown A, Dalpé G, Mathieu M, Kothary R. Cloning and characterization of the neural isoforms of human dystonin. Genomics. 1995;29:777-80 pubmed
    ..The mouse and human dystonin genes demonstrate the same spectrum of alternatively spliced products, and the amino acid sequences of the neural-specific exons in the mouse and human genes are over 96% identical. ..
  2. Hashimoto T, Wakabayashi T, Watanabe A, Kowa H, Hosoda R, Nakamura A, et al. CLAC: a novel Alzheimer amyloid plaque component derived from a transmembrane precursor, CLAC-P/collagen type XXV. EMBO J. 2002;21:1524-34 pubmed
    ..Both secreted and membrane-tethered forms of CLAC-P/collagen type XXV specifically bound to fibrillized Abeta, implicating these proteins in beta-amyloidogenesis and neuronal degeneration in AD. ..
  3. Liu Z, Shipley J, Vu T, Zhou X, Diaz L, Werb Z, et al. Gelatinase B-deficient mice are resistant to experimental bullous pemphigoid. J Exp Med. 1998;188:475-82 pubmed
    ..These results implicate neutrophil-derived gelatinase B in the pathogenesis of experimental BP and might lead to novel therapeutic strategies for BP. ..
  4. Leung C, Sun D, Liem R. The intermediate filament protein peripherin is the specific interaction partner of mouse BPAG1-n (dystonin) in neurons. J Cell Biol. 1999;144:435-46 pubmed
    ..Since peripherin and BPAG1-n also display similar expression patterns in the nervous system, we suggest that peripherin is the specific interaction partner of BPAG1-n in vivo. ..
  5. Osada Y, Hashimoto T, Nishimura A, Matsuo Y, Wakabayashi T, Iwatsubo T. CLAC binds to amyloid beta peptides through the positively charged amino acid cluster within the collagenous domain 1 and inhibits formation of amyloid fibrils. J Biol Chem. 2005;280:8596-605 pubmed
    ..These results suggest the anti-amyloidogenic roles of CLAC in the pathophysiology of Alzheimer's disease. ..
  6. Kowa H, Sakakura T, Matsuura Y, Wakabayashi T, Mann D, Duff K, et al. Mostly separate distributions of CLAC- versus Abeta40- or thioflavin S-reactivities in senile plaques reveal two distinct subpopulations of beta-amyloid deposits. Am J Pathol. 2004;165:273-81 pubmed
  7. Giudice G, Emery D, Diaz L. Cloning and primary structural analysis of the bullous pemphigoid autoantigen BP180. J Invest Dermatol. 1992;99:243-50 pubmed
    ..Autoantibody-mediated disruption of such an adhesive interaction may play a critical role in the development of sub-epidermal blisters in BP patients. ..
  8. Leung C, Zheng M, Prater S, Liem R. The BPAG1 locus: Alternative splicing produces multiple isoforms with distinct cytoskeletal linker domains, including predominant isoforms in neurons and muscles. J Cell Biol. 2001;154:691-7 pubmed
    ..The absence of BPAG1-a is the likely cause of sensory neurodegeneration in mutant mice. BPAG1-b is highly expressed in muscles, and has extra exons encoding a second IFBD between the plakin and SR-containing rod domains of BPAG1-a. ..
  9. Yang Y, Dowling J, Yu Q, Kouklis P, Cleveland D, Fuchs E. An essential cytoskeletal linker protein connecting actin microfilaments to intermediate filaments. Cell. 1996;86:655-65 pubmed
    ..In BPAG1 null mice, axonal architecture is markedly perturbed, consistent with a failure to tether neurofilaments to the actin cytoskeleton and underscoring the physiological relevance of this protein. ..
  10. Guo L, Degenstein L, Dowling J, Yu Q, Wollmann R, Perman B, et al. Gene targeting of BPAG1: abnormalities in mechanical strength and cell migration in stratified epithelia and neurologic degeneration. Cell. 1995;81:233-43 pubmed
    ..Unexpectedly, the mice also develop severe dystonia and sensory nerve degeneration typical of dystonia musculorum (dt/dt) mice. We show that in at least one other strain of dt/dt mice, BPAG1 gene is defective. ..
  11. Hopkinson S, Baker S, Jones J. Molecular genetic studies of a human epidermal autoantigen (the 180-kD bullous pemphigoid antigen/BP180): identification of functionally important sequences within the BP180 molecule and evidence for an interaction between BP180 and alpha 6 integrin. J Cell Biol. 1995;130:117-25 pubmed
    ..Perturbation of this latter domain by human bullous pemphigoid autoantibodies may explain the loss of epidermal cell-dermis attachment that characterizes the BP disease. ..
  12. Nishie W, Sawamura D, Goto M, Ito K, Shibaki A, McMillan J, et al. Humanization of autoantigen. Nat Med. 2007;13:378-83 pubmed
    ..This resulted in BP-like skin lesions and a human disease phenotype. Humanization of autoantigens is a new approach to the study of human autoimmune diseases. ..
  13. Kakuyama H, Söderberg L, Horigome K, Winblad B, Dahlqvist C, Naslund J, et al. CLAC binds to aggregated Abeta and Abeta fragments, and attenuates fibril elongation. Biochemistry. 2005;44:15602-9 pubmed
    ..Taken together, we suggest that CLAC becomes involved at an intermediate stage in the pathogenesis by binding to Abeta fibrils, including fibrils formed from peptides with truncated N- or C-termini, and thereby slows their growth. ..
  14. Wiche G, Becker B, Luber K, Weitzer G, Castanon M, Hauptmann R, et al. Cloning and sequencing of rat plectin indicates a 466-kD polypeptide chain with a three-domain structure based on a central alpha-helical coiled coil. J Cell Biol. 1991;114:83-99 pubmed
    ..This enabled us to tentatively map a sequence involved in plectin-vimentin and plectin-lamin B interactions to a restricted region of the rod domain. ..
  15. Liu Z, Diaz L, Swartz S, Troy J, Fairley J, Giudice G. Molecular mapping of a pathogenically relevant BP180 epitope associated with experimentally induced murine bullous pemphigoid. J Immunol. 1995;155:5449-54 pubmed
    ..These findings should help to elucidate the immunopathologic mechanisms responsible for human BP and HG and may have significant implications in the diagnosis and treatment of these autoimmune diseases. ..
  16. Kasperkiewicz M, Zillikens D. The pathophysiology of bullous pemphigoid. Clin Rev Allergy Immunol. 2007;33:67-77 pubmed
    ..The loss of cell-matrix adhesion is mediated by proteinases released by granulocytes. The increased knowledge of the pathophysiology of BP should facilitate the development of novel therapeutic strategies for this disease. ..
  17. Söderberg L, Kakuyama H, Möller A, Ito A, Winblad B, Tjernberg L, et al. Characterization of the Alzheimer's disease-associated CLAC protein and identification of an amyloid beta-peptide-binding site. J Biol Chem. 2005;280:1007-15 pubmed
    ..These findings may be useful for future therapeutic interventions aimed at finding compounds that modulate the binding of CLAC to Abeta deposits. ..
  18. Zone J, Taylor T, Hull C, Schmidt L, Meyer L. IgE basement membrane zone antibodies induce eosinophil infiltration and histological blisters in engrafted human skin on SCID mice. J Invest Dermatol. 2007;127:1167-74 pubmed
    ..Human skin grafts of control mice and normal mouse skin on the test mice and control mice did not develop any histological abnormalities. IgE antibodies to LABD97 recapitulate the histological inflammatory process seen in BP. ..
  19. Borradori L, Sonnenberg A. Hemidesmosomes: roles in adhesion, signaling and human diseases. Curr Opin Cell Biol. 1996;8:647-56 pubmed
    ..These multiprotein complexes appear not only to mediate cell adhesion, but also to transduce signals from the extracellular matrix to the cell interior that may profoundly modulate cell behavior. ..
  20. Borradori L, Koch P, Niessen C, Erkeland S, van Leusden M, Sonnenberg A. The localization of bullous pemphigoid antigen 180 (BP180) in hemidesmosomes is mediated by its cytoplasmic domain and seems to be regulated by the beta4 integrin subunit. J Cell Biol. 1997;136:1333-47 pubmed
    ..These results indicate that sequences within the cytoplasmic domain of beta4 determine the subcellular distribution of BP180. ..
  21. Chen R, Fairley J, Zhao M, Giudice G, Zillikens D, Diaz L, et al. 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. J Immunol. 2002;169:3987-92 pubmed
    ..MCs play a major role in neutrophil recruitment into the dermis. Furthermore, Mphi-mediated neutrophil infiltration depends on MC activation/degranulation. ..
  22. Söderberg L, Dahlqvist C, Kakuyama H, Thyberg J, Ito A, Winblad B, et al. Collagenous Alzheimer amyloid plaque component assembles amyloid fibrils into protease resistant aggregates. FEBS J. 2005;272:2231-6 pubmed
    ..Therefore, CLAC assembles Abeta fibrils into fibril bundles that have an increased resistance to proteases. We suggest that CLAC may act in a similar way in vivo. ..
  23. Okumura M, Yamakawa H, Ohara O, Owaribe K. Novel alternative splicings of BPAG1 (bullous pemphigoid antigen 1) including the domain structure closely related to MACF (microtubule actin cross-linking factor). J Biol Chem. 2002;277:6682-7 pubmed
    ..The results indicate that BPAG1 has three kinds of cytoskeletal binding domains and seems to play an important role in linking the different types of cytoskeletons. ..
  24. Forsell C, Björk B, Lilius L, Axelman K, Fabre S, Fratiglioni L, et al. Genetic association to the amyloid plaque associated protein gene COL25A1 in Alzheimer's disease. Neurobiol Aging. 2010;31:409-15 pubmed publisher
    ..Thus, in addition to the biochemical data, there is now genetic evidence of association between COL25A1 and risk for Alzheimer's disease. ..
  25. Tanaka T, Parry D, Klaus Kovtun V, Steinert P, Stanley J. Comparison of molecularly cloned bullous pemphigoid antigen to desmoplakin I confirms that they define a new family of cell adhesion junction plaque proteins. J Biol Chem. 1991;266:12555-9 pubmed
    ..S., Virata, L. A., Wagner, R. M., Angst, B. D., and Nilles, L. A. (1990) J. Biol. Chem. 265, 2603-2612) which show that BPA and desmoplakin I represent the first members of a new family of adhesion junction plaque proteins. ..
  26. Chen R, Ning G, Zhao M, Fleming M, Diaz L, Werb Z, et al. Mast cells play a key role in neutrophil recruitment in experimental bullous pemphigoid. J Clin Invest. 2001;108:1151-8 pubmed
    ..These findings provide the first direct evidence to our knowledge that MCs play an essential role in neutrophil recruitment during subepidermal blister formation in experimental BP. ..
  27. Li K, Tamai K, Tan E, Uitto J. Cloning of type XVII collagen. Complementary and genomic DNA sequences of mouse 180-kilodalton bullous pemphigoid antigen (BPAG2) predict an interrupted collagenous domain, a transmembrane segment, and unusual features in the 5'-end of the gene and t. J Biol Chem. 1993;268:8825-34 pubmed
    ..2 million years. In summary, we have cloned full-length mouse BPAG2 cDNA sequences that encode a collagenous polypeptide. We propose that this polypeptide be designated as the alpha 1-chain of type XVII collagen. ..
  28. Hirako Y, Usukura J, Nishizawa Y, Owaribe K. Demonstration of the molecular shape of BP180, a 180-kDa bullous pemphigoid antigen and its potential for trimer formation. J Biol Chem. 1996;271:13739-45 pubmed
    ..In conclusion, the present demonstration of the entire configuration of BP180, with a collagen-like trimer in its extracellular part, suggests that BP180 is one of the major components of anchoring filaments. ..
  29. Liu Z, Zhou X, Shapiro S, Shipley J, Twining S, Diaz L, et al. The serpin alpha1-proteinase inhibitor is a critical substrate for gelatinase B/MMP-9 in vivo. Cell. 2000;102:647-55 pubmed
    ..Excess NE produces lesions in GB -/- mice without cleaving alpha1-PI. Excess alpha1-PI phenocopies GB and NE deficiency in wild-type mice. ..
  30. Liu Z, Shapiro S, Zhou X, Twining S, Senior R, Giudice G, et al. A critical role for neutrophil elastase in experimental bullous pemphigoid. J Clin Invest. 2000;105:113-23 pubmed
    ..Finally, NE cleaved BP180 in vitro and in vivo. These results implicate NE directly in the dermal-epidermal cleavage induced by anti-BP180 antibodies in the experimental BP model. ..
  31. Sawamura D, Li K, Chu M, Uitto J. Human bullous pemphigoid antigen (BPAG1). Amino acid sequences deduced from cloned cDNAs predict biologically important peptide segments and protein domains. J Biol Chem. 1991;266:17784-90 pubmed
    ..These data suggest that BPAG1 may be a membrane-associated protein that plays a role in the attachment of basal keratinocytes to the underlying basement membrane. ..
  32. de Quatrebarbes J, Joly P. [Bullous pemphigoid]. Rev Prat. 2005;55:1165-8 pubmed
    ..Direct immunofluorescence shows linear IgG and/or C3 deposits along the dermal epidermal junction. In France and in Europe, most patients are now treated using topical steroid therapy (clobetasol propionate). ..
  33. Lee J, Fumimori T, Kurose K, Mori O, Hashimoto T. A case of bullous pemphigoid successfully treated by plasmapheresis: assessment of the change in titers of circulating antibodies by immunoblotting and enzyme-linked immunosorbent assay. J Dermatol. 2003;30:326-31 pubmed
    ..The reduction of titers of circulating autoantibodies in the sera correlated well with the decrease in the disease activity in both the first and second rounds of plasmapheresis treatment in this case. ..
  34. España A, del Olmo J, Marquina M, Sitaru C. [Mucous membrane pemphigoid: IgG and IgA antibodies against the BP180 antigen]. Actas Dermosifiliogr. 2005;96:365-70 pubmed
    ..Molecular biology techniques are very important to complement the diagnosis of MMP, especially when the results of hematoxylin-eosin or IF studies are not satisfactory for a diagnosis of MMP. ..
  35. Noe M, Messingham K, Brandt D, Andrews J, Fairley J. Pregnant women have increased incidence of IgE autoantibodies reactive with the skin and placental antigen BP180 (type XVII collagen). J Reprod Immunol. 2010;85:198-204 pubmed publisher
    ..We conclude that some pregnant women develop antibodies specific for BP180, and that these autoantibodies are capable of binding both CTB and the placental amnion, potentially affecting placental function. ..
  36. Yancey K. The pathophysiology of autoimmune blistering diseases. J Clin Invest. 2005;115:825-8 pubmed
  37. Di Zenzo G, Marazza G, Borradori L. Bullous pemphigoid: physiopathology, clinical features and management. Adv Dermatol. 2007;23:257-88 pubmed
  38. Tasanen K, Floeth M, Schumann H, Bruckner Tuderman L. Hemizygosity for a glycine substitution in collagen XVII: unfolding and degradation of the ectodomain. J Invest Dermatol. 2000;115:207-12 pubmed
    ..These observations show that the glycine substitution G633D in collagen XVII causes abnormal folding and susceptibility to degradation, and thus perturbs the physiologic adhesive functions of collagen XVII in the skin. ..
  39. Matsuoka Y, Yamada T, Seishima M, Hirako Y, Owaribe K, Kitajima Y. Transient translocation of hemidesmosomal bullous pemphigoid antigen 1 from cytosol to membrane fractions by 12-O-tetradecanoylphorbol-13-acetate treatment and Ca2+-switch in a human carcinoma cell line. J Dermatol Sci. 2001;27:206-14 pubmed
  40. Howard A, Dean D, Cooper S, Kirtshig G, Wojnarowska F. Circulating basement membrane zone antibodies are found in lichen sclerosus of the vulva. Australas J Dermatol. 2004;45:12-5 pubmed
    ..These antibodies may be a reflection of a tendency to produce autoantibodies or be relevant to pathogenesis. ..
  41. Murrell D, Pasmooij A, Pas H, Marr P, Klingberg S, Pfendner E, et al. Retrospective diagnosis of fatal BP180-deficient non-Herlitz junctional epidermolysis bullosa suggested by immunofluorescence (IF) antigen-mapping of parental carriers bearing enamel defects. J Invest Dermatol. 2007;127:1772-5 pubmed
  42. Pasmooij A, Pas H, Jansen G, Lemmink H, Jonkman M. Localized and generalized forms of blistering in junctional epidermolysis bullosa due to COL17A1 mutations in the Netherlands. Br J Dermatol. 2007;156:861-70 pubmed
    ..The data are important for genetic counselling at early age when the clinical phenotype is not yet clear. ..
  43. Tang H, Chaffotte A, Thacher S. Structural analysis of the predicted coiled-coil rod domain of the cytoplasmic bullous pemphigoid antigen (BPAG1). Empirical localization of the N-terminal globular domain-rod boundary. J Biol Chem. 1996;271:9716-22 pubmed
    ..These findings support the existence of a common domain structure in the N-terminal regions of the BPAG1/DPI/plectin family. ..
  44. Kromminga A, Sitaru C, Hagel C, Herzog S, Zillikens D. Development of an ELISA for the detection of autoantibodies to BP230. Clin Immunol. 2004;111:146-52 pubmed
    ..This ELISA provides a useful tool for the detection of autoantibodies to BP230 in BP and other diseases associated with an autoimmune response to this protein. ..
  45. Chan L, Lapiere J, Chen M, Traczyk T, Mancini A, Paller A, et al. Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type VII collagen. Arch Dermatol. 1999;135:569-73 pubmed
    ..Possible pathogenic mechanisms in this patient's clinical diseases include provocation of organ-specific disease (bullous disease) by systemic autoimmunity (lupus) and the "epitope spreading" immune phenomenon. ..
  46. Ishiura N, Fujimoto M, Watanabe R, Nakashima H, Kuwano Y, Yazawa N, et al. Serum levels of IgE anti-BP180 and anti-BP230 autoantibodies in patients with bullous pemphigoid. J Dermatol Sci. 2008;49:153-61 pubmed
    ..IgE autoantibodies to BP180 and BP230 are detected at high frequencies in BP. IgE anti-BP230 antibodies may have a role in attracting eosinophils to the skin lesions. ..
  47. Zimina E, Bruckner Tuderman L, Franzke C. Shedding of collagen XVII ectodomain depends on plasma membrane microenvironment. J Biol Chem. 2005;280:34019-24 pubmed
    ..These data deliver the first evidence for the role of plasma membrane lipid organization in the regulation of collagen XVII shedding and, therefore, in the regulation of keratinocyte migration and differentiation. ..
  48. Cooke N, Jenkinson H, Wojnarowska F, McKenna K, Alderdice J. Coexistence of psoriasis and linear IgA disease in a patient with recent herpes zoster infection. Clin Exp Dermatol. 2005;30:643-5 pubmed
    ..This is an interesting case that we believe is an example of epitope spreading in the development of autoimmune subepidermal bullous diseases. ..
  49. Chen W, Rogatsky I, Garabedian M. MED14 and MED1 differentially regulate target-specific gene activation by the glucocorticoid receptor. Mol Endocrinol. 2006;20:560-72 pubmed
    ..Our findings suggest that modulation of the Mediator subunit activities would provide a mechanism for promoter selectivity by GR. ..
  50. Feliciani C, Caldarola G, Kneisel A, Podstawa E, Pfütze M, Pfutzner W, et al. IgG autoantibody reactivity against bullous pemphigoid (BP) 180 and BP230 in elderly patients with pruritic dermatoses. Br J Dermatol. 2009;161:306-12 pubmed publisher
    ..IgG-mediated autoimmunity against the intracellular BP230 may facilitate a chronic, inflammatory response eventually leading to full-blown BP which is presumably associated with IgG against BP180. ..
  51. Amagai M. [Autoimmune blistering diseases]. Nihon Rinsho. 2005;63 Suppl 5:540-6 pubmed
  52. Franzke C, Bruckner P, Bruckner Tuderman L. Collagenous transmembrane proteins: recent insights into biology and pathology. J Biol Chem. 2005;280:4005-8 pubmed
  53. Figueira E, Crotty A, Challinor C, Coroneo M, Murrell D. Granulation tissue in the eyelid margin and conjunctiva in junctional epidermolysis bullosa with features of laryngo-onycho-cutaneous syndrome. Clin Exp Ophthalmol. 2007;35:163-6 pubmed
    ..On investigation, our patient had a combination of a unique mutation in LAMA3 and the mutation I17N in LAMA3a, providing further evidence that laryngo-onycho-cutaneous syndrome is a variant of junctional EB. ..
  54. Patsatsi A, Vyzantiadis T, Devliotou Panagiotidou D, Chrysomallis F, Sotiriadis D. Detection of anti-BP180NC16a and anti-BP230 autoantibodies in blister fluid of patients with bullous pemphigoid: the first survey in Greece. Clin Exp Dermatol. 2008;33:183-5 pubmed
    ..Our results indicate that blister-fluid examination by ELISA can be a useful tool to diagnose bullous pemphigoid, especially in elderly patients who refuse biopsy or have poor venous access. ..
  55. Shimbo T, Tanemura A, Yamazaki T, Tamai K, Katayama I, Kaneda Y. Serum anti-BPAG1 auto-antibody is a novel marker for human melanoma. PLoS ONE. 2010;5:e10566 pubmed publisher
    ..Here, we report anti-BPAG1 auto-antibodies as a promising marker for the diagnosis of melanoma, and we discuss the significance of the detection of such auto-antibodies in cancer biology and patients. ..
  56. Koster J, Geerts D, Favre B, Borradori L, Sonnenberg A. Analysis of the interactions between BP180, BP230, plectin and the integrin alpha6beta4 important for hemidesmosome assembly. J Cell Sci. 2003;116:387-99 pubmed
  57. Huilaja L, Hurskainen T, Autio Harmainen H, Hofmann S, Sormunen R, Rasanen J, et al. Pemphigoid gestationis autoantigen, transmembrane collagen XVII, promotes the migration of cytotrophoblastic cells of placenta and is a structural component of fetal membranes. Matrix Biol. 2008;27:190-200 pubmed
    ..Although the exact pathomechanism of PG is still largely unknown, the clinical symptoms of PG are initiated after the expression of collagen XVII in placenta during the first trimester of pregnancy. ..
  58. Esteve E. [Pemphigoid gestationis]. Presse Med. 2010;39:1071-5 pubmed publisher
    ..Management in a specialized setting is therefore necessary. Recurrence is possible during subsequent pregnancies. ..
  59. Schachter M, Brieva J, Jones J, Zillikens D, Skrobek C, Chan L. Pemphigoid nodularis associated with autoantibodies to the NC16A domain of BP180 and a hyperproliferative integrin profile. J Am Acad Dermatol. 2001;45:747-54 pubmed
  60. Nomura K, Sugawara T, Sato T, Sawamura D, Hashimoto I, Sugita Y, et al. Expression of laminin, type IV procollagen and 230 kDa bullous pemphigoid antigen genes by keratinocytes and fibroblasts in culture: application of the polymerase chain reaction for detection of small amounts of messenger RNA. Arch Dermatol Res. 1994;286:408-13 pubmed
  61. Izumi R, Fujimoto M, Yazawa N, Nakashima H, Asashima N, Watanabe R, et al. Bullous pemphigoid positive for anti-BP180 and anti-laminin 5 antibodies in a patient with graft-vs-host disease. J Am Acad Dermatol. 2007;56:S94-7 pubmed
    ..Immune disturbance as well as destruction of basal epidermal cells and base membrane by GVHD may result in the induction of autoimmune blistering diseases with unusual clinical and laboratory manifestations. ..
  62. Zillikens D, Herzele K, Georgi M, Schmidt E, Chimanovitch I, Schumann H, et al. Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP1801. J Invest Dermatol. 1999;113:947-53 pubmed
    ..Our results demonstrate that IgA autoantibodies from a subset of linear IgA disease patients react with the same sites on BP180 that are targeted by IgG autoantibodies in bullous pemphigoid. ..
  63. Georgi M, Scheckenbach C, Kromminga A, Partscht K, Messer G, Brocker E, et al. Mapping of epitopes on the BP180 ectodomain targeted by IgA and IgG autoantibodies in patients with the lamina lucida-type of linear IgA disease. Arch Dermatol Res. 2001;293:109-14 pubmed
    ..This is also the major region recognized by autoantibodies in patients with CP. This finding correlates with the observation that there may be significant overlap of the clinical and immunopathological findings in LAD and CP. ..
  64. Favre B, Fontao L, Koster J, Shafaatian R, Jaunin F, Saurat J, et al. The hemidesmosomal protein bullous pemphigoid antigen 1 and the integrin beta 4 subunit bind to ERBIN. Molecular cloning of multiple alternative splice variants of ERBIN and analysis of their tissue expression. J Biol Chem. 2001;276:32427-36 pubmed
  65. Nykvist P, Tasanen K, Viitasalo T, Kapyla J, Jokinen J, Bruckner Tuderman L, et al. The cell adhesion domain of type XVII collagen promotes integrin-mediated cell spreading by a novel mechanism. J Biol Chem. 2001;276:38673-9 pubmed
    ..After being shed from the cell surface, it may support keratinocyte spreading and migration. ..
  66. Lai F, Sheu H, Lee J, Cheng C, Chen W. Vesicular pemphigoid with circulating autoantibodies against 230-kDa and 180-kDa proteins, and additional autoantibodies against 97-kDa and 45-kDa proteins. Int J Dermatol. 2007;46:206-9 pubmed
    ..Treatment with systemic corticosteroid, minocycline and antihistamines plus topical steroid resulted in moderate improvement. A few vesicles, however, continued to appear intermittently during the past year of treatment. ..
  67. Parikka M, Nissinen L, Kainulainen T, Bruckner Tuderman L, Salo T, Heino J, et al. Collagen XVII promotes integrin-mediated squamous cell carcinoma transmigration--a novel role for alphaIIb integrin and tirofiban. Exp Cell Res. 2006;312:1431-8 pubmed
    ..Together, these data demonstrate a novel interaction between collagen XVII and alphaIIb integrin and also suggest a possibility to use tirofiban to inhibit the invasion and progression of alphaIIb expressing SCC tumors. ..
  68. Seppänen A, Autio Harmainen H, Alafuzoff I, Särkioja T, Veijola J, Hurskainen T, et al. Collagen XVII is expressed in human CNS neurons. Matrix Biol. 2006;25:185-8 pubmed
    ..We propose that collagen XVII may be involved in the pathogenesis of various disorders affecting neuronal migration or synaptic plasticity. ..
  69. Uitto J, Pulkkinen L. Molecular complexity of the cutaneous basement membrane zone. Mol Biol Rep. 1996;23:35-46 pubmed
    ..Thus, genetic lesions resulting in abnormalities in any part of this network could result in a blistering skin disease, such as epidermolysis bullosa. ..
  70. Xu L, O Toole E, Olivry T, Hernandez C, Peng J, Chen M, et al. Molecular cloning of canine bullous pemphigoid antigen 2 cDNA and immunomapping of NC16A domain by canine bullous pemphigoid autoantibodies. Biochim Biophys Acta. 2000;1500:97-107 pubmed
    ..The cloning of the cDNA encoding this disease-associated protein may allow us to develop a canine model in dissecting the immunopathologic mechanism underlying bullous pemphigoid. ..
  71. Hirako Y, Yoshino K, Zillikens D, Owaribe K. Extracellular cleavage of bullous pemphigoid antigen 180/type XVII collagen and its involvement in hemidesmosomal disassembly. J Biochem. 2003;133:197-206 pubmed
    ..Our observations demonstrate that the cleavage of BP180 occurring within the NC16A domain is mediated by a membrane-associated metalloprotease and suggest a possible involvement of the cleavage in hemidesmosomal disassembly...
  72. Desai N, Allen J, Ali I, Venning V, Wojnarowska F. Autoantibodies to basement membrane proteins BP180 and BP230 are commonly detected in normal subjects by immunoblotting. Australas J Dermatol. 2008;49:137-41 pubmed publisher
    ..In conclusion, significant numbers of normal healthy subjects have circulating autoantibodies to basement membrane proteins, chiefly BP180 detectable by immunoblot, but these do not bind the NC16A domain. ..
  73. Atzori L, Deidda S, Aste N. Enzyme-linked immunosorbent assay in autoimmune blistering diseases: preliminary experience of the Dermatology Department of Cagliari. G Ital Dermatol Venereol. 2008;143:1-8 pubmed
    ..More over, autoantibodies titre correlate with disease severity, and is useful to monitor treatment response. ..
  74. Iwata H, Kamio N, Aoyama Y, Yamamoto Y, Hirako Y, Owaribe K, et al. IgG from patients with bullous pemphigoid depletes cultured keratinocytes of the 180-kDa bullous pemphigoid antigen (type XVII collagen) and weakens cell attachment. J Invest Dermatol. 2009;129:919-26 pubmed publisher
    ..In the presence of BP180 deficiency, inflammation generated by BP180 immune-complex formation might then tear the weakened lamina lucida, and this could lead to generation of the BP-specific split at the lamina lucida. ..
  75. Pas H, Kloosterhuis G, de Jong M, Jonkman M. False-negative results in immunoblot assay of serum IgA antibodies reactive with the 180-kDa bullous pemphigoid antigen: the importance of primary incubation temperature. Br J Dermatol. 2001;145:986-9 pubmed
    ..Primary incubation at room temperature may result in false-negative results in the IgA-BP180 immunoblot assay. ..