19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa.

19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa. Research Abstract Details 

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19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa. Abstract Text:

A murine monoclonal antibody (19-DEJ-1) was recently produced that recognizes a unique antigenic epitope of human skin basement membrane localized to the midlamina lucida exclusively in those areas bordered by overlying hemidesmosomes. To determine whether the antigen defined by 19-DEJ-1 is normally expressed in one or more forms of epidermolysis bullosa (EB) known to have structural and antigenic defects in skin basement membrane, we examined by indirect immunofluorescence 46 specimens of clinically normal skin from 43 patients representing each of the four forms of inherited EB (simplex, 15 patients; junctional, nine patients; dominant dystrophic, seven patients; and recessive dystrophic, 15 patients). All nine junctional EB specimens revealed absent 19-DEJ-1 binding; similarly, seven of 15 and one of six recessive and dominant dystrophic EB skin specimens, respectively, showed lack of antibody recognition. In contrast, binding was normal in 15 of 15 EB simplex specimens and five of six dominant dystrophic EB specimens. Absent binding of 19-DEJ-1 to junctional EB skin correlates well with the localization of this antigen to the region of the midlamina lucida. Its absence in approximately 50% of patients with recessive dystrophic EB further suggests, for the first time, that an inherited defect in basement membrane is shared in patients with junctional and some recessive dystrophic EB. The difference in site of skin cleavage in the latter two disorders gives additional support to the hypothesis that 19-DEJ-1 recognizes a structure, such as the anchoring filament, which both bridges the entire basement membrane and contributes to epidermal-dermal adhesion.

19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa. Publishing Authors By Initials

For similar integumentary system: skin research abstracts see: integumentary system: skin research

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19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa. Journal Published:

PUBLICATION TYPE: Research Support, U.S. Gov't,

Journal: Archives of dermatology

VOLUME: 125

Page Numbers: 520-3

Journal Abbreviation: Arch Dermatol

ISSN: 0003-987X

DAY: 17

MONTH: Apr

YEAR: 1989

19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa. Information

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LANGUAGE: eng

NlmUniqueID: 372433

19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa. Keywords Mesh Terms:

KEYWORDS: Skin

MESH TERMS: ultrastructure

Chemical & Substance for Abstract: 19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa. Information

Substance Name: Antibodies, Monoclonal

Registry Number: 0

Grant and Affiliation Information for 19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa.

AFFILIATION: Department of Dermatology, University of Alabama, Birmingham School of Medicine 35294.

Country: UNITED STATES

AGENCY: United States NIAMS

GRANT: 5RO1 AR36629

ACRONYM: AR

MEDLINETA: Arch Dermatol

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