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Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis
Open accessAbstract
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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Cited by
The Oslo definitions for coeliac disease and related terms
Ludvigsson, J. F., Leffler, D. A., Bai, J. C., Biagi, F., Fasano, A., Green, P. H. R., Hadjivassiliou, M., Kaukinen, K., Kelly, C. P., Leonard, J. N., et al. (2013) · Gut · doi:10.1136/gutjnl-2011-301346 · Cited by 1,721
The Effects of a Smoking Cessation Intervention on 14.5-Year Mortality: A Randomized Clinical Trial
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Gluten sensitivity: from gut to brain
Hadjivassiliou, M., Sanders, D. S., Grünewald, R. A., Woodroofe, N., Boscolo, S., & Aeschlimann, D. (2010) · The Lancet Neurology · doi:10.1016/s1474-4422(09)70290-x · Cited by 404
Narrative Review: Celiac Disease: Understanding a Complex Autoimmune Disorder
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Metadata
- Authors
- Sárdy, M., Kárpáti, S., Merkl, B., Paulsson, M., & Smyth, N.
- Venue
- The Journal of Experimental Medicine
- Publisher
- Rockefeller University Press
- Published
- 2002-03-18
- Volume
- 195
- Issue
- 6
- Pages
- 747-757
- ISSN
- 0022-1007 (ISSN-L)
- 1540-9538
- OpenAlex
- W2116288844
- Cited by
- 517
- References
- 25
- FWCI
- 11.30
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Sárdy, M., Kárpáti, S., Merkl, B., Paulsson, M., & Smyth, N. (2002). Epidermal Transglutaminase (TGase 3) Is the Autoantigen of Dermatitis Herpetiformis. The Journal of Experimental Medicine. https://doi.org/10.1084/jem.20011299
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