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Titre du document / Document title

Type VII collagen, anchoring fibrils, and epidermolysis bullosa

Auteur(s) / Author(s)

BURGESON R. E. ;

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

Harvard medical school, Massachusetts gen. hosp., cutaneous biology res. cent., Charlestown MA 02129, ETATS-UNIS

Résumé / Abstract

The anchoring fibrils at the dermal-epidermal junction have been well characterized as ultrastructural entities. From their appearance, it was proposed that they fortified the attachment of the epidermis to the dermis. This hypothesized function was strengthened by observations indicating that the anchoring fibrils were abnormal, diminished, or absent from individuals with dystrophic epidermolysis bullosa. Therefore, characterization of the molecular constituents of the anchoring fibrils and their interactions with other basement membrane and dermal components might lead to identification of the gene defects underlying at least some forms of epidermolysis bullosa. Type VII collagen was identified as the protein component of anchoring fibrils in 1986. Since then, the major characteristics of the molecule have been described

Revue / Journal Title

Journal of investigative dermatology    ISSN  0022-202X   CODEN JIDEAE 

Source / Source

Congrès
JSID, SID, and ESDR. Tricontinental meeting No2, Kyoto , JAPON (28/10/1993)
1993, vol. 101, no 3, pp. 387-500 (46 ref.), pp. 252-255

Langue / Language

Anglais

Editeur / Publisher

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

Mots-clés anglais / English Keywords

Dystrophic epidermolysis bullosa

;

Collagen

;

Fibril

;

Anchoring

;

Gene expression

;

Skin

;

Pathogenesis

;

Review

;

Human

;

Basement membrane

;

Skin disease

;

Bullous dermatosis

;

Genetic disease

;

Mots-clés français / French Keywords

Epidermolyse bulleuse dystrophique

;

Collagène

;

Fibrille

;

Ancrage

;

Expression génique

;

Peau

;

Pathogénie

;

Article synthèse

;

Collagène type VII

;

Homme

;

Lame basale

;

Peau pathologie

;

Dermatose bulleuse

;

Maladie héréditaire

;

Mots-clés espagnols / Spanish Keywords

Epidermólisis bulosa distrófica

;

Colágeno

;

Fibrilla

;

Anclaje

;

Expresión genética

;

Piel

;

Patogenia

;

Artículo síntesis

;

Hombre

;

Lámina basal

;

Piel patología

;

Dermatosis bulosa

;

Enfermedad hereditaria

;

Localisation / Location

INIST-CNRS, Cote INIST : 3194, 35400004784980.0020

Nº notice refdoc (ud4) : 4900196



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