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Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis

Gluten sensitivity typically presents as celiac disease, a common chronic small intestinal disorder. However, in certain individuals it is associated with dermatitis herpetiformis, a blistering skin disease characterized by granular IgA deposits in the papillary dermis. While tissue transglutaminase...

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Autores principales: Sárdy, Miklós, Kárpáti, Sarolta, Merkl, Barbara, Paulsson, Mats, Smyth, Neil
Formato: Texto
Lenguaje:English
Publicado: The Rockefeller University Press 2002
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2193738/
https://www.ncbi.nlm.nih.gov/pubmed/11901200
http://dx.doi.org/10.1084/jem.20011299
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author Sárdy, Miklós
Kárpáti, Sarolta
Merkl, Barbara
Paulsson, Mats
Smyth, Neil
author_facet Sárdy, Miklós
Kárpáti, Sarolta
Merkl, Barbara
Paulsson, Mats
Smyth, Neil
author_sort Sárdy, Miklós
collection PubMed
description Gluten sensitivity typically presents as celiac disease, a common chronic small intestinal disorder. However, in certain individuals it is associated with dermatitis herpetiformis, a blistering skin disease characterized by granular IgA deposits in the papillary dermis. While tissue transglutaminase has been implicated as the major autoantigen of gluten sensitive disease, there has been no explanation as to why this condition appears in two distinct forms. Here we show that while sera from patients with either form of gluten sensitive disease react both with tissue transglutaminase and the related enzyme epidermal (type 3) transglutaminase, antibodies in patients having dermatitis herpetiformis show a markedly higher avidity for epidermal transglutaminase. Further, these patients have an antibody population specific for this enzyme. We also show that the IgA precipitates in the papillary dermis of patients with dermatitis herpetiformis, the defining signs of the disease, contain epidermal transglutaminase, but not tissue transglutaminase or keratinocyte transglutaminase. These findings demonstrate that epidermal transglutaminase, rather than tissue transglutaminase, is the dominant autoantigen in dermatitis herpetiformis and explain why skin symptoms appear in a proportion of patients having gluten sensitive disease.
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spelling pubmed-21937382008-04-14 Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis Sárdy, Miklós Kárpáti, Sarolta Merkl, Barbara Paulsson, Mats Smyth, Neil J Exp Med Original Article Gluten sensitivity typically presents as celiac disease, a common chronic small intestinal disorder. However, in certain individuals it is associated with dermatitis herpetiformis, a blistering skin disease characterized by granular IgA deposits in the papillary dermis. While tissue transglutaminase has been implicated as the major autoantigen of gluten sensitive disease, there has been no explanation as to why this condition appears in two distinct forms. Here we show that while sera from patients with either form of gluten sensitive disease react both with tissue transglutaminase and the related enzyme epidermal (type 3) transglutaminase, antibodies in patients having dermatitis herpetiformis show a markedly higher avidity for epidermal transglutaminase. Further, these patients have an antibody population specific for this enzyme. We also show that the IgA precipitates in the papillary dermis of patients with dermatitis herpetiformis, the defining signs of the disease, contain epidermal transglutaminase, but not tissue transglutaminase or keratinocyte transglutaminase. These findings demonstrate that epidermal transglutaminase, rather than tissue transglutaminase, is the dominant autoantigen in dermatitis herpetiformis and explain why skin symptoms appear in a proportion of patients having gluten sensitive disease. The Rockefeller University Press 2002-03-18 /pmc/articles/PMC2193738/ /pubmed/11901200 http://dx.doi.org/10.1084/jem.20011299 Text en Copyright © 2002, The Rockefeller University Press This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/4.0/).
spellingShingle Original Article
Sárdy, Miklós
Kárpáti, Sarolta
Merkl, Barbara
Paulsson, Mats
Smyth, Neil
Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis
title Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis
title_full Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis
title_fullStr Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis
title_full_unstemmed Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis
title_short Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis
title_sort epidermal transglutaminase (tgase 3) is the autoantigen of dermatitis herpetiformis
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2193738/
https://www.ncbi.nlm.nih.gov/pubmed/11901200
http://dx.doi.org/10.1084/jem.20011299
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