Epidermal transglutaminase (TGase 3) Is the autoantigen of dermatitis herpetiformis

Sárdy, Miklós, Karpati, Sarolta, Merkl, Barbara, Paulsson, Mats and Smyth, Neil (2002) Epidermal transglutaminase (TGase 3) Is the autoantigen of dermatitis herpetiformis The Journal of Experimental Medicine, 195, (6), pp. 747-757. (doi:10.1084/jem.20011299).


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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.

Item Type: Article
Digital Object Identifier (DOI): doi:10.1084/jem.20011299
ISSNs: 0022-1007 (print)
Related URLs:
Keywords: gluten sensitive enteropathy, celiac disease, IgA, immune complex, skin
Subjects: R Medicine > RL Dermatology
Q Science > QR Microbiology > QR180 Immunology
ePrint ID: 37532
Date :
Date Event
18 March 2002Published
Date Deposited: 25 May 2006
Last Modified: 16 Apr 2017 22:03
Further Information:Google Scholar
URI: http://eprints.soton.ac.uk/id/eprint/37532

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