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Titre du document / Document title

Structural variations in anchoring fibrils in dystrophic epidermolysis bullosa : correlation with type VII collagen expression

Auteur(s) / Author(s)

MCGRATH J. A. (1) ; ISHIDA-YAMAMOTO A. (1) ; O'GRADY A. (1) ; LEIGH I. M. ; EADY R. A. J. (1) ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

(1) Saint Thomas's hosp., united medical dental schools, dep. cell pathology, London SE1 7EH, ROYAUME-UNI

Résumé / Abstract

Vystrophic epidermolysis bullosa is characterized by various abnormalities of anchoring fibrils, which are mainly composed of type VII collagen, at the dermal-epidermal junction. To define these changes more clearly, we examined skin samples from 22 patients with different forms of dystrophic epidermolysis bullosa by pre-embedding immunoelectron microscopy using an antibody (LH 7:2) that binds to the NC-1 globular domain of type VII collagen, followed by 1 nm colloidal gold-labeled secondary antibodies and subsequent silver enhancement. In dominant dystrophic epidermolysis bullosa cases, there was only a slight but variable reduction in the immunolabeling density on anchoring fibrils and on the lamina densa, in parts similar to normal human skin

Revue / Journal Title

Journal of investigative dermatology    ISSN  0022-202X   CODEN JIDEAE 

Source / Source

Congrès
European Society for Dermatological Research. Annual scientific meeting, Amsterdam , PAYS-BAS (03/04/1993)
1993, vol. 100, no 4, pp. 435-487 (45 ref.), pp. 366-372

Langue / Language

Anglais

Editeur / Publisher

Nature Publishing Group, New York, NY, ETATS-UNIS  (1938) (Revue)

Mots-clés anglais / English Keywords

Dystrophic epidermolysis bullosa

;

Molecular form

;

Dermoepidermal junction

;

Anchoring

;

Fibril

;

Collagen

;

Glycoproteins

;

Ultrastructure

;

Immunohistochemistry

;

Pathology

;

Exploration

;

Human

;

Skin disease

;

Bullous dermatosis

;

Genetic disease

;

Mots-clés français / French Keywords

Epidermolyse bulleuse dystrophique

;

Forme moléculaire

;

Jonction dermoépidermique

;

Ancrage

;

Fibrille

;

Collagène

;

Glycoprotéine

;

Ultrastructure

;

Immunohistochimie

;

Anatomopathologie

;

Exploration

;

Collagène type VII

;

Homme

;

Peau pathologie

;

Dermatose bulleuse

;

Maladie héréditaire

;

Mots-clés espagnols / Spanish Keywords

Epidermólisis bulosa distrófica

;

Forma molecular

;

Unión dermoepidérmica

;

Anclaje

;

Fibrilla

;

Colágeno

;

Glicoproteina

;

Ultraestructura

;

Inmunohistoquímica

;

Anatomía patológica

;

Exploración

;

Hombre

;

Piel patología

;

Dermatosis bulosa

;

Enfermedad hereditaria

;

Localisation / Location

INIST-CNRS, Cote INIST : 3194, 35400003355576.0040

Nº notice refdoc (ud4) : 4680348



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