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Antigenic expression of integrin α6β4 in junctional epidermolysis bullosa
Author(s) -
PHILLIPS R.J.,
APLIN J.D.,
LAKE B.D.
Publication year - 1994
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/j.1365-2559.1994.tb00578.x
Subject(s) - junctional epidermolysis bullosa (veterinary medicine) , epidermolysis bullosa , hemidesmosome , epidermolysis bullosa simplex , antibody , medicine , pathology , papillary dermis , epitope , integrin , immunohistochemistry , dermis , immunology , biology , basement membrane , laminin , receptor , microbiology and biotechnology , extracellular matrix
Integrin α6β4 is a major component of hemidesmosomes, considered to play a central role in the adhesion of basal epidermal cells to the underlying dermis. It is therefore of considerable interest in the study of the aetiology of inherited blistering disorders. We have examined the immunohistochemical characteristics of skin from 16 patients with epidermolysis bullosa using two antibodies directed against epitopes on the β4 subunit of αβ4 integrin (G71, 3E1), one antibody directed against an epitope on the α6 subunit (GoH3), GB3 an antibody for nicein, and LH7.2, an anti‐collagen type VII antibody. All 10 patients with junctional epidermolysis bullosa showed markedly reduced or no immunoreactivity with G71. These patients included two with GB3‐positive junctional epidermolysis bullosa associated with pyloric atresia, and four with other subtypes. By contrast, five patients with dystrophic epidermolysis bullosa and one patient with epidermolysis bullosa simplex showed normal immunoreactivity with G71. In this study, G71 is shown to have a high specificity and sensitivity for the diagnosis of junctional epidermolysis bullosa. Immunoreactivity with 3E1 and GoH3 was normal in most patients, consistent with published reports showing normal immunoreactivity with other β4 and α6 subunit antibodies. The data suggest a modification of the β4 subunit of integrin α6β4 at the dermo‐epidermal junction in junctional epidermolysis bullosa.