Altered expression of a new antigen of the dermal-epidermal junction (NU-T2 DEJ Ag) in junctional epidermolysis bullosa. 1995

G Tadini, and J Kanitakis, and R Cavalli, and D Schmitt, and S Cambiaghi, and E Berti
Center for Inherited Cutaneous Diseases, IRCCS Ospedale Maggiore, University of Milan, Italy.

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.

UI MeSH Term Description Entries
D004817 Epidermis The external, nonvascular layer of the skin. It is made up, from within outward, of five layers of EPITHELIUM: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).
D005455 Fluorescent Antibody Technique Test for tissue antigen using either a direct method, by conjugation of antibody with fluorescent dye (FLUORESCENT ANTIBODY TECHNIQUE, DIRECT) or an indirect method, by formation of antigen-antibody complex which is then labeled with fluorescein-conjugated anti-immunoglobulin antibody (FLUORESCENT ANTIBODY TECHNIQUE, INDIRECT). The tissue is then examined by fluorescence microscopy. Antinuclear Antibody Test, Fluorescent,Coon's Technique,Fluorescent Antinuclear Antibody Test,Fluorescent Protein Tracing,Immunofluorescence Technique,Coon's Technic,Fluorescent Antibody Technic,Immunofluorescence,Immunofluorescence Technic,Antibody Technic, Fluorescent,Antibody Technics, Fluorescent,Antibody Technique, Fluorescent,Antibody Techniques, Fluorescent,Coon Technic,Coon Technique,Coons Technic,Coons Technique,Fluorescent Antibody Technics,Fluorescent Antibody Techniques,Fluorescent Protein Tracings,Immunofluorescence Technics,Immunofluorescence Techniques,Protein Tracing, Fluorescent,Protein Tracings, Fluorescent,Technic, Coon's,Technic, Fluorescent Antibody,Technic, Immunofluorescence,Technics, Fluorescent Antibody,Technics, Immunofluorescence,Technique, Coon's,Technique, Fluorescent Antibody,Technique, Immunofluorescence,Techniques, Fluorescent Antibody,Techniques, Immunofluorescence,Tracing, Fluorescent Protein,Tracings, Fluorescent Protein
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000941 Antigens Substances that are recognized by the immune system and induce an immune reaction. Antigen
D012867 Skin The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D016108 Epidermolysis Bullosa Dystrophica Form of epidermolysis bullosa characterized by atrophy of blistered areas, severe scarring, and nail changes. It is most often present at birth or in early infancy and occurs in both autosomal dominant and recessive forms. All forms of dystrophic epidermolysis bullosa result from mutations in COLLAGEN TYPE VII, a major component fibrils of BASEMENT MEMBRANE and EPIDERMIS. Cockayne-Touraine Disease,Epidermolysis Bullosa, Dystrophic,Hallopeau-Siemens Disease,Cockayne-Touraine Type Epidermolysis Bullosa,Dystrophic Epidermolysis Bullosa,Dystrophic Epidermolysis Bullosa, Autosomal Recessive,Epidermolysis Bullosa Dystrophica, Autosomal Recessive,Epidermolysis Bullosa Dystrophica, Cockayne-Touraine Type,Epidermolysis Bullosa Dystrophica, Dominant,Epidermolysis Bullosa Dystrophica, Hallopeau-Siemens Type,Epidermolysis Bullosa Dystrophica, Recessive,Bullosa Dystrophica, Epidermolysis,Bullosa Dystrophicas, Epidermolysis,Bullosa, Dystrophic Epidermolysis,Bullosas, Dystrophic Epidermolysis,Cockayne Touraine Disease,Cockayne Touraine Type Epidermolysis Bullosa,Dystrophic Epidermolysis Bullosas,Dystrophica, Epidermolysis Bullosa,Dystrophicas, Epidermolysis Bullosa,Epidermolysis Bullosa Dystrophica, Cockayne Touraine Type,Epidermolysis Bullosa Dystrophica, Hallopeau Siemens Type,Epidermolysis Bullosa Dystrophicas,Epidermolysis Bullosas, Dystrophic,Hallopeau Siemens Disease
D016109 Epidermolysis Bullosa, Junctional Form of epidermolysis bullosa having onset at birth or during the neonatal period and transmitted through autosomal recessive inheritance. It is characterized by generalized blister formation, extensive denudation, and separation and cleavage of the basal cell plasma membranes from the basement membrane. Epidermolysis Bullosa Letalis,Herlitz Disease,Junctional Epidermolysis Bullosa,Lethal Junctional Epidermolysis Bullosa,Epidermolysis Bullosa Junctionalis, Disentis Type,Epidermolysis Bullosa Junctionalis, Herlitz Type,Epidermolysis Bullosa Junctionalis, Progressive,Epidermolysis Bullosa Junctionalis, Severe Nonlethal,Epidermolysis Bullosa Progressiva,Epidermolysis Bullosa, Generalized Atrophic Benign,Epidermolysis Bullosa, Junctional, Herlitz Type,Epidermolysis Bullosa, Junctional, Herlitz-Pearson Type,Herlitz's Disease,Herlitz-Pearson Type Epidermolysis Bullosa,Herlitz-Pearson-Type Epidermolysis Bullosa,Bullosa Letali, Epidermolysis,Bullosa Letalis, Epidermolysis,Bullosa, Herlitz-Pearson-Type Epidermolysis,Disease, Herlitz,Disease, Herlitz's,Epidermolysis Bullosa Letali,Epidermolysis Bullosa, Herlitz-Pearson-Type,Herlitz Pearson Type Epidermolysis Bullosa,Herlitzs Disease,Letali, Epidermolysis Bullosa,Letalis, Epidermolysis Bullosa
D016110 Epidermolysis Bullosa Simplex A form of epidermolysis bullosa characterized by serous bullae that heal without scarring. Mutations in the genes that encode KERATIN-5 and KERATIN-14 have been associated with several subtypes of epidermolysis bullosa simplex. Epidermolysis Bullosa Herpetiformis Dowling-Meara,Epidermolysis Bullosa Simplex Kobner,Weber-Cockayne Syndrome,EBS, Acral Form,EBS, Generalized,EBS-DM,Epidermolysis Bullosa Herpetiformis Dowling Meara,Epidermolysis Bullosa Herpetiformis, Dowling-Meara,Epidermolysis Bullosa Herpetiformis, Dowling-Meara Type,Epidermolysis Bullosa Simplex, Cockayne-Touraine Type,Epidermolysis Bullosa Simplex, Dowling-Meara Type,Epidermolysis Bullosa Simplex, Generalized,Epidermolysis Bullosa Simplex, Koebner Type,Epidermolysis Bullosa Simplex, Localized,Epidermolysis Bullosa Simplex, Weber-Cockayne Type,Epidermolysis Bullosa of Hands and Feet,Weber-Cockayne Type Epidermolysis Bullosa Simplex,Acral Form EBS,Acral Form EBSs,Bullosa Simplices, Epidermolysis,EBSs, Acral Form,EBSs, Generalized,Epidermolysis Bullosa Herpetiformis, Dowling Meara,Epidermolysis Bullosa Herpetiformis, Dowling Meara Type,Epidermolysis Bullosa Simplex, Cockayne Touraine Type,Epidermolysis Bullosa Simplex, Dowling Meara Type,Epidermolysis Bullosa Simplex, Weber Cockayne Type,Epidermolysis Bullosa Simplices,Generalized EBS,Generalized EBSs,Weber Cockayne Syndrome,Weber Cockayne Type Epidermolysis Bullosa Simplex

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