Epidermolysis Bullosa Acquisita Antigen is the Globular Carboxyl Terminus of Type VII Procollagen

Overview

Journal J Clin Invest

Specialty General Medicine

Date 1988 Mar 1

PMID 3278005

Citations 44

Authors

D T Woodley

R E Burgeson

G Lunstrum

L Bruckner-Tuderman

M J Reese

R A Briggaman

Affiliations

Soon will be listed here.

Abstract

Epidermolysis bullosa acquisita (EBA) is a severe, chronic blistering disease of the skin. EBA patients have circulating and tissue-bound autoantibodies to a large (Mr = 290,000) macromolecule that is localized within the basement membrane zone between the epidermis and dermis of skin, the site of blister formation. The "EBA antigen" is known to be distinct from laminin, heparan sulfate proteoglycan, fibronectin, the bullous pemphigoid antigen, elastin, and collagen types I, II, III, IV, and V. Sera from patients with EBA, two monoclonal antibodies to the EBA antigen, and a monoclonal antibody to the carboxyl terminus of type VII procollagen identically label human amnion and skin by immunofluorescent and immunoelectron microscopy. Western immunoblots of the EBA antigen extracted from skin and of type VII procollagen labeled with the above sera and antibodies are identical. None of the sera or antibodies labels Western blots of pepsinized type VII collagen which is missing the globular amino and carboxyl terminal domains. These data show that the EBA antigen is the carboxyl terminus of type VII procollagen.

Citing Articles

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Oral Alterations in Heritable Epidermolysis Bullosa: A Clinical Study and Literature Review.

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