Cleavage of BP180, a 180-kDa Bullous Pemphigoid Antigen, Yields a 120-kDa Collagenous Extracellular Polypeptide

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Demonstration of the molecular shape of BP180, a 180-kDa bullous pemphigoid antigen and its potential for trimer formation.

The 180-kDa bullous pemphigoid antigen (BP180) is a hemidesmosomal transmembrane glycoprotein comprising interrupted collagen domains in its extracellular part. BP180 is also termed type XVII collagen. But the question of whether it actually takes a collagen-like triple helical conformation in vivo has remained unanswered. Using a monoclonal antibody, we found that a subpopulation of BP180 loca...

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Genomic organization of collagenous domains and chromosomal assignment of human 180-kDa bullous pemphigoid antigen-2, a novel collagen of stratified squamous epithelium.

We have recently isolated a 1.0-kilobase (kb) cDNA encoding 180-kDa bullous pemphigoid antigen (BPAG2), an autoantigen in blistering skin disease, bullous pemphigoid (Giudice, G. J., Squiquera, H. L., Elias, P. M., and Diaz, L. (1991) J. Clin. Invest. 87, 734-738). The deduced amino acid sequence identified two collagenous domains characterized by Gly-X-Y repeats. In this study we have elucidat...

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A Case of Chronic Bullous Disease of Childhood That Was Reactive to the Antigen of 120 kDa (LAD-1).

Chronic bullous disease of childhood (CBDC) is an autoimmune blistering disease that is characterized by Immunoglobulin A (IgA) deposits at the basement membrane zone. IgA autoantibodies (aAbs) from the serum of patients with CBDC react with antigens of 97 kDa (LABD97) and 120 kDa (LAD-1), and both of which are fragments of the extracellular domain of bullous pemphigoid 180 (BP180, type XVII co...

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Mucous membrane pemphigoid with IgG autoantibodies to the 120-kDa ectodomain of type XVII collagen (BP180/linear IgA dermatosis antigen) in a patient with idiopathic thrombocytopenic purpura.

© 2015 The Authors. doi: 10.2340/00015555-1964 Journal Compilation © 2015 Acta Dermato-Venereologica. ISSN 0001-5555 Mucous membrane pemphigoid (MMP) is an autoimmune subepidermal blistering disease involving primarily the mucosae and occasionally the skin (1–3). We report here a case of a Japanese male patient with idiopathic thrombocytopenic purpura (ITP) who later developed MMP with IgG auto...

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BP180 as the common autoantigen in blistering diseases with different clinical phenotypes.

Bullous pemphigoid antigen 180 (BP180, type XVII collagen) is a transmembrane hemidesmosomal glycoprotein of basal keratinocytes that spans the lamina lucida of the dermal-epidermal junction. Five autoimmune subepidermal blistering diseases are associated with an immune response to BP180, including bullous pemphigoid (BP), pemphigoid gestations (PG), cicatricial pemphigoid (CP), lichen planus p...

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ژورنال

عنوان ژورنال: Journal of Biological Chemistry

سال: 1998

ISSN: 0021-9258

DOI: 10.1074/jbc.273.16.9711