Skip to main content
Log in

Altered expression of a new antigen of the dermal-epidermal junction (NU-T2 DEJ Ag) in junctional epidermolysis bullosa

  • Original Paper
  • Published:
Archives of Dermatological Research Aims and scope Submit manuscript

Abstract

NU-T2 antigen (Ag) is a new and recently described antigen of the dermal-epidermal junction, recognized by an anti-CD1b monoclonal antibody denominated NU-T2. We studied NU-T2 Ag expression in junctional epidermolysis bullosa (13 patients) and in other forms of hereditary epidermolysis bullosa (23 patients), comparing the results with nicein expression. In junctional epidermolysis bullosa gravis type no differences were found between the expression of NU-T2 and nicein, both being negative in bullous as well as in non-bullous skin. Interestingly, in mitis type junctional epidermolysis bullosa, NU-T2 Ag was found to be absent or reduced in five of six patients both in lesional and in uncleaved skin. When compared with nicein expression, clearcut differences were found, further suggesting that these two antigens are different. These data confirm that NU-T2 Ag is a novel epitope of the dermal-epidermal junction, probably relevant in dermal-epidermal cohesion, and it could be responsible, together with nicein, 19-DEJ-1 and other adhesion molecules, for the different subtypes of junctional epidermolysis bullosa. Finally, NU-T2 monoclonal antibody is a new relevant tool for the diagnosis, classification, and prenatal diagnosis of junctional epidermolysis bullosa.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Fine JD, Bauer EA, Briggaman RA, Carter DM, Eady RAJ, Esterly NB, Holbrook KA, Hurwitz S, Johnson L, Lin AN, Pearsons R, Sybert P (1991) Revised clinical and laboratory criteria for subtypes of inherited epidermolysis bullosa. A consensus report by the Subcommittee on Diagnosis and Classification of the National Epidermolysis Bullosa Registry. J Am Acad Dermatol 24: 119–135

    PubMed  Google Scholar 

  2. Tidman MJ, Eady RAJ (1986) Hemidesmosomes heterogeneity in junctional epidermolysis bullosa revealed by morphometric analysis. J Invest Dermatol 86: 51–56

    PubMed  Google Scholar 

  3. Krueger JG, Lin AN, Leong I, Carter DM (1991) Junctional epidermolysis bullosa keratinocytes in culture display adhesive, structural, and functional abnormalities. J Invest Dermatol 97: 849–861

    PubMed  Google Scholar 

  4. Verrando P, Hsi BL, Yeh CJ, Pisani A, Serleys N, Ortonne JP (1987) Monoclonal antibody GB3, a new probe for the study of human basement membranes and hemidesmosomes. Exp Cell Res 170: 116–128

    PubMed  Google Scholar 

  5. Burgeson RE, Chiquet M, Deutzmann R, Ekblom P, Engel J, Kleinman H, Martin GR, Meneguzzi G, Paulsson M, Sanes J, Timpl R, Tryggvason K, Yamada Y, Yurchenko PD (1994) A new nomenclature for the laminins. Matrix Biol 14: 209–211

    PubMed  Google Scholar 

  6. Pulkkinen L, Christiano A, Airenne T, Haakana H, Tryggvasson K, Uitto J (1994) Mutations in the γ2 chain gene (LAMC2) of kalinin/laminin5 in the junctional forms of epidermolysis bullosa. Nat Genet 6: 293–298

    PubMed  Google Scholar 

  7. Aberdam D, Galliano MF, Vailly J, Pulkkinen L, Bonifas J, Christiano A, Trygvasson K, Uitto J, Epstein EH Jr, Ortonne JP, Meneguzzi G (1994) Herlitz's junctional epidermolysis bullosa is linked to mutations in the gene (LAMC2) for the γ2 subunit of nicein/kalinin (Laminin5). Nat Genet 6: 299–304

    PubMed  Google Scholar 

  8. Baudoin C, Miquel C, Gagnoux-Palacios L, Pulkkinen L, Christiano A, Uitto J, Tadini G, Ortonne JP, Meneguzzi G (1994) A novel homozygous nonsense mutation in the LAMC2 gen in patients with the Herlitz junctional epidermolysis bullosa. Hum Mol Genet 3 (10): 1109–1110

    PubMed  Google Scholar 

  9. Fine JD, Horiguchi Y, Couchman JR (1989) 19DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa. Arch Dermatol 125: 520–523

    PubMed  Google Scholar 

  10. Jonkman FM, Jong MC de, Heeres K, Sonnenberg A (1992) Expression of Integrin α6-β4 in junctional epidermolysis bullosa. J Invest Dermatol 99: 489–496

    PubMed  Google Scholar 

  11. Cattoretti G, Berti E, Mancuso A, D'Amato L, Schirò R, Soligo D, Delia D (1989) An MHC class I related family of antigens with widespread distribution on resting and activated cells. In: McMichael AJ (ed) Leucocyte typing III. Oxford University Press, Oxford, 89–92

    Google Scholar 

  12. Kanitakis J, Zambruno G, Wang YZ, Roche P, Berti E, Schmitt D (1993) A novel antigen of the dermal-epidermal junction defined by an anti-CD1b monoclonal antibody (NU-T2). Arch Dermatol Res 285: pp 313–321

    PubMed  Google Scholar 

  13. Furue M, Nindl M, Kawabe K, Nakamura K, Ishibashi Y, Sagawa K (1992) Epitopes for CD1a, CD1b, and CD1c antigens are differentially mapped on Langerhans cells, dermal dendritic cells, keratinocytes, and basement membrane zone in human skin. J Am Acad Dermatol 27: 419–426

    PubMed  Google Scholar 

  14. Fine JD, Breathnach SM, Hintner H, Katz SI (1984) KF-1 monoclonal antibody defines a specific basement membrane antigen defect in dystrophic forms of epidermolysis bullosa. J Invest Dermatol 82: 35–38

    PubMed  Google Scholar 

  15. Heagerty AH, Kennedy AR, Leigh IM, Purkis P, Eady RA (1986) Identification of an epidermal basement membrane defect in recessive forms of dystrophic epidermolysis bullosa by LH7: 2 monoclonal antibody: use in diagnosis. Br J Dermatol 115: 125–131

    PubMed  Google Scholar 

  16. Cavalli R, Cambiaghi S, Brusasco A, Tadini G, Ermacora E, Berti E (1992) Impiego di markers immunologici nella diagnosi delle epidermolisi bollose ereditarie. G Ital Dermatol Venereol 127: 135–142

    Google Scholar 

  17. Verrando P, Blanchet-Bardon C, Pisani A, Thomas L, Cambazard R, Eady RAJ, Schofield O, Ortonne JP (1991) Monoclonal antibody GB3 defines a widespread defect of several basement membranes and a keratinocyte dysfunction in patient with lethal junctional epidermolysis bullosa. Lab Invest 64: 85–92

    PubMed  Google Scholar 

  18. Nazzaro V, Nicolini U, De Luca L, Berti E, Caputo R (1990) Prenatal diagnosis of junctional epidermolysis bullosa associated with pyloric atresia. J Med Genet 27: 244–248

    PubMed  Google Scholar 

  19. Fine JD (1990) 19-DEJ-1, a monoclonal antibody to the hemidesmosome-anchoring filament complex, is the only reliable immunohistochemical probe for all major forms of junctional epidermolysis bullosa. Arch Dermatol 126: 1187–1190

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Tadini, G., Kanitakis, J., Cavalli, R. et al. Altered expression of a new antigen of the dermal-epidermal junction (NU-T2 DEJ Ag) in junctional epidermolysis bullosa. Arch Dermatol Res 287, 699–704 (1995). https://doi.org/10.1007/BF01105792

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01105792

Key words

Navigation