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Titolo:
DEFECTIVE INTEGRIN ALPHA-6-BETA-4 EXPRESSION IN THE SKIN OF PATIENTS WITH JUNCTIONAL EPIDERMOLYSIS-BULLOSA AND PYLORIC ATRESIA
Autore:
BROWN TA; GIL SG; SYBERT VP; LESTRINGANT GG; TADINI G; CAPUTO R; CARTER WG;
Indirizzi:
FRED HUTCHINSON CANC RES CTR,DIV BASIC SCI,1124 COLUMBIA ST,A3-015 SEATTLE WA 98104 UNIV WASHINGTON,SCH MED,DEPT PEDIAT,DIV MED GENET SEATTLE WA 98195 UNIV WASHINGTON,SCH MED,DEPT PEDIAT,DIV DERMATOL SEATTLE WA 98195 UNIV WASHINGTON,SCH MED,DEPT MED SEATTLE WA 98195 CHILDRENS HOSP & MED CTR SEATTLE WA 98105 TAWAM HOSP ABU ZABY U ARAB EMIRATES UNIV MILAN,DEPT CLIN & PEDIAT DERMATOL I-20122 MILAN ITALY UNIV WASHINGTON,DEPT PATHOBIOL SEATTLE WA 98195
Titolo Testata:
Journal of investigative dermatology
fascicolo: 3, volume: 107, anno: 1996,
pagine: 384 - 391
SICI:
0022-202X(1996)107:3<384:DIAEIT>2.0.ZU;2-F
Fonte:
ISI
Lingua:
ENG
Soggetto:
BASEMENT-MEMBRANE ADHESION; MONOCLONAL-ANTIBODY; PEMPHIGOID ANTIGEN; CELL-ADHESION; MOLECULAR-GENETICS; LAMININ CHAIN; HEMIDESMOSOMES; COMPONENT; ALPHA-3-BETA-1; EPILIGRIN;
Keywords:
VESICULOBULLOUS SKIN DISEASES; HEMIDESMOSOMES; CELL ADHESION; EPIDERMIS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
59
Recensione:
Indirizzi per estratti:
Citazione:
T.A. Brown et al., "DEFECTIVE INTEGRIN ALPHA-6-BETA-4 EXPRESSION IN THE SKIN OF PATIENTS WITH JUNCTIONAL EPIDERMOLYSIS-BULLOSA AND PYLORIC ATRESIA", Journal of investigative dermatology, 107(3), 1996, pp. 384-391

Abstract

Junctional epidermolysis bullosa (JEB) is a heterogeneous group of rare inherited skin disorders in which defects in cell adhesion components cause incomplete formation of hemidesmosomes. We have immunohistochemically examined the skin from ten JEB patients (JEB gravis, n = 4; JEB mitis, n = 3; JEB plus pyloric atresia [JEB/PA], n = 3) using monoclonal antibodies specific for the integrin adhesion receptors alpha 3 beta 1 and alpha 6 beta 4, and for the alpha 3, beta 3, and gamma 2 subunits of the basement membrane ligand, laminin 5. Consistent with ourprevious reports, only the JEB gravis patients without associated pyloric atresia expressed reduced or absent epitopes for laminin-5 subunits in their epidermal basement membrane. In contrast, all three JEB/PAcases showed abnormalities in integrin alpha 6 beta 4, but not in Laminin 5, expression in their basal epidermal cells, Integrin beta 4 subunit was undetectable in the biopsied epidermis of these JEB/PA individuals using five different monoclonal antibodies that recognize both intra- and extracellular epitopes. The absence of the beta 4 subunit inthe epidermis of JEB/PA specimens was confirmed by Western blot analysis of tissue extracts, Epidermal expression of the integrin alpha 6 subunit was variable in the JEB/PA patients. Abnormal integrin alpha 6 beta 4 expression map define the subset of JEB cases with pyloric atresia. These results strongly implicate a functional role for alpha 6 beta 4 in the formation of complete hemidesmosomes and in stable adhesion of basal keratinocytes to the basement membrane in vivo.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 03/12/20 alle ore 12:29:54