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Gene Review

DST  -  dystonin

Homo sapiens

Synonyms: 230 kDa bullous pemphigoid antigen, 230/240 kDa bullous pemphigoid antigen, BP230, BP240, BPA, ...
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Disease relevance of DST


Psychiatry related information on DST


High impact information on DST

  • Moreover, BPAG1 can functionally account for the extraordinary stability of axonal microtubules necessary for transport over long distances [11].
  • These novel findings emphasize the molecular heterogeneity of this group of genodermatoses, and attest to the importance of BPAG2 in maintaining adhesion between the epidermis and the dermis [12].
  • 180-kD bullous pemphigoid antigen (BP180) is deficient in generalized atrophic benign epidermolysis bullosa [13].
  • The human 230-kD bullous pemphigoid antigen gene (BPAG1). Exon-intron organization and identification of regulatory tissue specific elements in the promoter region [14].
  • Detailed knowledge of the structure and regulation of the BPAG1 gene will aid in further elucidation of diseases affecting the cutaneous basement membrane zone [14].

Chemical compound and disease context of DST


Biological context of DST


Anatomical context of DST

  • Previously, we reported that the expression of 230-kDa bullous pemphigoid antigen (BPAG1) gene, which is strictly restricted to basal keratinocytes, is transcriptionally suppressed by IFN-gamma, but the contribution of IRFs in such suppression is still unclear [2].
  • 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 [22].
  • BPA and desmoplakin I, a desmosome plaque protein, show remarkable sequence and structural homology [23].
  • However, in contrast to its high homology with BPAG1 at the NH(2)-terminal, the COOH-terminal structure of MACF, including a microtubule binding domain, resembles dystrophin rather than plakins [22].
  • Intriguingly, in one non-epidermal tumour line (a pancreatic cell line termed FG), the 280 kDa polypeptide appears to be the only product of the BPA gene [24].

Associations of DST with chemical compounds


Physical interactions of DST


Regulatory relationships of DST


Other interactions of DST


Analytical, diagnostic and therapeutic context of DST

  • Furthermore, expression of the HD-associated structural proteins, bullous pemphigoid antigens 1 (BPAG 1) and 2 (BPAG 2), was demonstrated in NHBE cells by immunofluorescence microscopy and immunoblot analyses [38].
  • Moreover, we have confirmed the co-expression of the 230 and 280 kDa polypeptides in these cells by immunoblotting analyses using a monoclonal antibody preparation directed against a polypeptide encoded by sequence common to both mRNAs transcribed from the BPA gene [24].
  • These cells also synthesize BPA in vitro, and immunoprecipitation from metabolically labeled cultures revealed a 220 kD polypeptide under reducing conditions [39].
  • Subsequent immunoelectron microscopy using both peroxidase and colloidal gold labeling techniques with patients' sera or IgG, revealed that BPA is associated with hemidesmosomes--putative adhesion structures at the BMZ, based on their similarity in ultrastructure to desmosomes [39].
  • Two cDNAs, a 0.45-kb PCR product synthesized with human keratinocyte RNA as template and a 2.2-kb cDNA isolated from human keratinocyte lambda gt11 library, were utilized for chromosomal in situ hybridizations to establish the genomic location of the BPAG1 gene [40].


  1. The N terminus of the transmembrane protein BP180 interacts with the N-terminal domain of BP230, thereby mediating keratin cytoskeleton anchorage to the cell surface at the site of the hemidesmosome. Hopkinson, S.B., Jones, J.C. Mol. Biol. Cell (2000) [Pubmed]
  2. Transcriptional regulation of the 230-kDa bullous pemphigoid antigen gene expression by interferon regulatory factor 1 and interferon regulatory factor 2 in normal human epidermal keratinocytes. Odanagi, M., Kikuchi, Y., Yamazaki, T., Kaneko, T., Nakano, H., Tamai, K., Uitto, J., Vitto, J., Hanada, K. Exp. Dermatol. (2004) [Pubmed]
  3. 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. Tang, H.Y., Chaffotte, A.F., Thacher, S.M. J. Biol. Chem. (1996) [Pubmed]
  4. Bullous pemphigoid antigen: cDNA cloning, cellular expression, and evidence for polymorphism of the human gene. Sawamura, D., Li, K.H., Nomura, K., Sugita, Y., Christiano, A.M., Uitto, J. J. Invest. Dermatol. (1991) [Pubmed]
  5. Isolation of a human epidermal cDNA corresponding to the 180-kD autoantigen recognized by bullous pemphigoid and herpes gestationis sera. Immunolocalization of this protein to the hemidesmosome. Diaz, L.A., Ratrie, H., Saunders, W.S., Futamura, S., Squiquera, H.L., Anhalt, G.J., Giudice, G.J. J. Clin. Invest. (1990) [Pubmed]
  6. The dex/CRH test and the DST in patients with bipolar disorder and major depressive disorder. Watson, S., Gallagher, P., Smith, M.S., Nicol Ferrier, I., Young, A.H. Psychoneuroendocrinology (2007) [Pubmed]
  7. The DST and organic affective disorder. Fahs, J.J. The American journal of psychiatry. (1985) [Pubmed]
  8. Influence of hospital admission on DST results in patients with panic disorder. Szádóczky, E., Rihmer, Z., Arató, M. The American journal of psychiatry. (1986) [Pubmed]
  9. Relationship of serum TSH concentration and antithyroid antibodies to diagnosis and DST response in psychiatric inpatients. Haggerty, J.J., Simon, J.S., Evans, D.L., Nemeroff, C.B. The American journal of psychiatry. (1987) [Pubmed]
  10. Lack of association between cortisol hypersecretion and nonsuppression on the DST in patients with Alzheimer's disease. Miller, A.H., Sastry, G., Speranza, A.J., Lawlor, B.A., Mohs, R.C., Ryan, T.M., Gabriel, S.M., Serby, M., Schmeidler, J., Davis, K.L. The American journal of psychiatry. (1994) [Pubmed]
  11. Integrators of the cytoskeleton that stabilize microtubules. Yang, Y., Bauer, C., Strasser, G., Wollman, R., Julien, J.P., Fuchs, E. Cell (1999) [Pubmed]
  12. Mutations in the 180-kD bullous pemphigoid antigen (BPAG2), a hemidesmosomal transmembrane collagen (COL17A1), in generalized atrophic benign epidermolysis bullosa. McGrath, J.A., Gatalica, B., Christiano, A.M., Li, K., Owaribe, K., McMillan, J.R., Eady, R.A., Uitto, J. Nat. Genet. (1995) [Pubmed]
  13. 180-kD bullous pemphigoid antigen (BP180) is deficient in generalized atrophic benign epidermolysis bullosa. Jonkman, M.F., de Jong, M.C., Heeres, K., Pas, H.H., van der Meer, J.B., Owaribe, K., Martinez de Velasco, A.M., Niessen, C.M., Sonnenberg, A. J. Clin. Invest. (1995) [Pubmed]
  14. The human 230-kD bullous pemphigoid antigen gene (BPAG1). Exon-intron organization and identification of regulatory tissue specific elements in the promoter region. Tamai, K., Sawamura, D., Do, H.C., Tamai, Y., Li, K., Uitto, J. J. Clin. Invest. (1993) [Pubmed]
  15. Transforming growth factor-beta up-regulates the expression of the genes for beta 4 integrin and bullous pemphigoid antigens (BPAG1 and BPAG2) in normal and transformed human keratinocytes. Sollberg, S., Ryynänen, J., Olsen, D.R., Uitto, J. J. Invest. Dermatol. (1992) [Pubmed]
  16. Cortisol suppression and circadian rhythm in endogenous depression: a preliminary report. Lee, M.A., Taylor, M.A. Biol. Psychiatry (1983) [Pubmed]
  17. The activity of piperacillin/tazobactam against clinical isolates collected in 20 UK centres and the design of a disc test for susceptibility testing. Reeves, D.S., Holt, H.A., Bywater, M.J., MacGowan, A.P. J. Antimicrob. Chemother. (1993) [Pubmed]
  18. Clonidine in the treatment of mixed bipolar disorder. Kontaxakis, V., Markianos, M., Markidis, M., Stefanis, C. Acta psychiatrica Scandinavica. (1989) [Pubmed]
  19. Evaluation of 5-fluorouracil applicability by multi-point collagen gel droplet embedded drug sensitivity test. Ochiai, T., Nishimura, K., Noguchi, H., Kitajima, M., Tsuruoka, Y., Takahashi, Y. Oncol. Rep. (2005) [Pubmed]
  20. 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]
  21. Human bullous pemphigoid antigen (BPAG1). Amino acid sequences deduced from cloned cDNAs predict biologically important peptide segments and protein domains. Sawamura, D., Li, K., Chu, M.L., Uitto, J. J. Biol. Chem. (1991) [Pubmed]
  22. Novel alternative splicings of BPAG1 (bullous pemphigoid antigen 1) including the domain structure closely related to MACF (microtubule actin cross-linking factor). Okumura, M., Yamakawa, H., Ohara, O., Owaribe, K. J. Biol. Chem. (2002) [Pubmed]
  23. Comparison of molecularly cloned bullous pemphigoid antigen to desmoplakin I confirms that they define a new family of cell adhesion junction plaque proteins. Tanaka, T., Parry, D.A., Klaus-Kovtun, V., Steinert, P.M., Stanley, J.R. J. Biol. Chem. (1991) [Pubmed]
  24. Identification of a second protein product of the gene encoding a human epidermal autoantigen. Hopkinson, S.B., Jones, J.C. Biochem. J. (1994) [Pubmed]
  25. Disruption of ERBB2IP is not associated with dystrophic epidermolysis bullosa in both father and son carrying a balanced 5;13 translocation. Stefanova, M., Zemke, K., Dimitrov, B., Has, C., Kern, J.S., Bruckner-Tuderman, L., Kutsche, K. J. Invest. Dermatol. (2005) [Pubmed]
  26. Assessment of the validity of the dexamethasone/corticotropin-releasing hormone test Comment on: Watson, S., Gallagher, P., Smith, M.S., Ferrier, I.N., Young, A.H., 2006. The dex/CRH test-Is it better than the DST? Psychoneuroendocrinology 31, 889-894. Eede, F.V., Claes, S.J. Psychoneuroendocrinology (2007) [Pubmed]
  27. HD4, a 180 kDa bullous pemphigoid antigen, is a major transmembrane glycoprotein of the hemidesmosome. Nishizawa, Y., Uematsu, J., Owaribe, K. J. Biochem. (1993) [Pubmed]
  28. A novel homozygous point mutation in the COL17A1 gene in a Chinese family with generalized atrophic benign epidermolysis bullosa. Wu, Y., Li, G., Zhu, X. J. Dermatol. Sci. (2002) [Pubmed]
  29. 180-kD bullous pemphigoid antigen/type XVII collagen: tissue-specific expression and molecular interactions with keratin 18. Aho, S., Uitto, J. J. Cell. Biochem. (1999) [Pubmed]
  30. Precise ultrastructural localization of in vivo deposited IgG antibodies in fresh perilesional skin of patients with bullous pemphigoid. Sato, M., Shimizu, H., Ishiko, A., Masunaga, T., Akiyama, M., Hashimoto, T., Kitajima, Y., Nishikawa, T. Br. J. Dermatol. (1998) [Pubmed]
  31. Specificity of binding of the plectin actin-binding domain to beta4 integrin. Litjens, S.H., Koster, J., Kuikman, I., van Wilpe, S., de Pereda, J.M., Sonnenberg, A. Mol. Biol. Cell (2003) [Pubmed]
  32. Coexistence of pemphigus foliaceus and bullous pemphigoid. Demonstration of autoantibodies that bind to both the pemphigus foliaceus antigen complex and the bullous pemphigoid antigen. Korman, N.J., Stanley, J.R., Woodley, D.T. Archives of dermatology. (1991) [Pubmed]
  33. Red cell antigens and renal transplantation. Duguid, J.K., Hillis, A.N., Bone, J.M. Transplantation (1990) [Pubmed]
  34. Increased adrenocorticotropin suppression following dexamethasone administration in sexually abused adolescents with posttraumatic stress disorder. Duval, F., Crocq, M.A., Guillon, M.S., Mokrani, M.C., Monreal, J., Bailey, P., Macher, J.P. Psychoneuroendocrinology (2004) [Pubmed]
  35. Catechol-O-methyltransferase activity in cultured human skin fibroblasts from controls and patients with dystonia musculorum deformans. Breakefield, X.O., Braverman, M., Riker, D.K., Giller, E.L. J. Neurosci. Res. (1981) [Pubmed]
  36. Genomic organization of collagenous domains and chromosomal assignment of human 180-kDa bullous pemphigoid antigen-2, a novel collagen of stratified squamous epithelium. Li, K.H., Sawamura, D., Giudice, G.J., Diaz, L.A., Mattei, M.G., Chu, M.L., Uitto, J. J. Biol. Chem. (1991) [Pubmed]
  37. Analysis of the interactions between BP180, BP230, plectin and the integrin alpha6beta4 important for hemidesmosome assembly. Koster, J., Geerts, D., Favre, B., Borradori, L., Sonnenberg, A. J. Cell. Sci. (2003) [Pubmed]
  38. Human bronchial epithelial cells secrete laminin 5, express hemidesmosomal proteins, and assemble hemidesmosomes. Michelson, P.H., Tigue, M., Jones, J.C. J. Histochem. Cytochem. (2000) [Pubmed]
  39. Bullous pemphigoid antigen localization suggests an intracellular association with hemidesmosomes. Westgate, G.E., Weaver, A.C., Couchman, J.R. J. Invest. Dermatol. (1985) [Pubmed]
  40. Bullous pemphigoid antigen (BPAG1): cDNA cloning and mapping of the gene to the short arm of human chromosome 6. Sawamura, D., Nomura, K., Sugita, Y., Mattei, M.G., Chu, M.L., Knowlton, R., Uitto, J. Genomics (1990) [Pubmed]
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