The protease inhibitor alpha-2-macroglobulin-like-1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human.
<h4>Background</h4>Paraneoplastic pemphigus (PNP) is a devastating autoimmune blistering disease, involving mucocutaneous and internal organs, and associated with underlying neoplasms. PNP is characterized by the production of autoantibodies targeting proteins of the plakin and cadherin...
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| Format: | Article |
| Language: | English |
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Public Library of Science (PLoS)
2010-08-01
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| Series: | PLoS ONE |
| Online Access: | https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/20805888/pdf/?tool=EBI |
| _version_ | 1818845487851110400 |
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| author | Isabelle Schepens Fabienne Jaunin Nadja Begre Ursula Läderach Katrin Marcus Takashi Hashimoto Bertrand Favre Luca Borradori |
| author_facet | Isabelle Schepens Fabienne Jaunin Nadja Begre Ursula Läderach Katrin Marcus Takashi Hashimoto Bertrand Favre Luca Borradori |
| author_sort | Isabelle Schepens |
| collection | DOAJ |
| description | <h4>Background</h4>Paraneoplastic pemphigus (PNP) is a devastating autoimmune blistering disease, involving mucocutaneous and internal organs, and associated with underlying neoplasms. PNP is characterized by the production of autoantibodies targeting proteins of the plakin and cadherin families involved in maintenance of cell architecture and tissue cohesion. Nevertheless, the identity of an antigen of Mr 170,000 (p170), thought to be critical in PNP pathogenesis, has remained unknown.<h4>Methodology/principal findings</h4>Using an immunoprecipitation and mass spectrometry based approach, we identified p170 as alpha-2-macroglobuline-like-1, a broad range protease inhibitor expressed in stratified epithelia and other tissues damaged in the PNP disease course. We demonstrate that 10 PNP sera recognize alpha-2-macroglobuline-like-1 (A2ML1), while none of the control sera obtained from patients with bullous pemphigoid, pemphigus vulgaris, pemphigus foliaceus and normal subjects does.<h4>Conclusions/significance</h4>Our study unravels a broad range protease inhibitor as a new class of target antigens in a paraneoplastic autoimmune multiorgan syndrome and opens a new challenging investigation avenue for a better understanding of PNP pathogenesis. |
| first_indexed | 2024-12-19T05:30:27Z |
| format | Article |
| id | doaj.art-14cb1e83950043eda1b12d1e32f82835 |
| institution | Directory Open Access Journal |
| issn | 1932-6203 |
| language | English |
| last_indexed | 2024-12-19T05:30:27Z |
| publishDate | 2010-08-01 |
| publisher | Public Library of Science (PLoS) |
| record_format | Article |
| series | PLoS ONE |
| spelling | doaj.art-14cb1e83950043eda1b12d1e32f828352022-12-21T20:34:15ZengPublic Library of Science (PLoS)PLoS ONE1932-62032010-08-0158e1225010.1371/journal.pone.0012250The protease inhibitor alpha-2-macroglobulin-like-1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human.Isabelle SchepensFabienne JauninNadja BegreUrsula LäderachKatrin MarcusTakashi HashimotoBertrand FavreLuca Borradori<h4>Background</h4>Paraneoplastic pemphigus (PNP) is a devastating autoimmune blistering disease, involving mucocutaneous and internal organs, and associated with underlying neoplasms. PNP is characterized by the production of autoantibodies targeting proteins of the plakin and cadherin families involved in maintenance of cell architecture and tissue cohesion. Nevertheless, the identity of an antigen of Mr 170,000 (p170), thought to be critical in PNP pathogenesis, has remained unknown.<h4>Methodology/principal findings</h4>Using an immunoprecipitation and mass spectrometry based approach, we identified p170 as alpha-2-macroglobuline-like-1, a broad range protease inhibitor expressed in stratified epithelia and other tissues damaged in the PNP disease course. We demonstrate that 10 PNP sera recognize alpha-2-macroglobuline-like-1 (A2ML1), while none of the control sera obtained from patients with bullous pemphigoid, pemphigus vulgaris, pemphigus foliaceus and normal subjects does.<h4>Conclusions/significance</h4>Our study unravels a broad range protease inhibitor as a new class of target antigens in a paraneoplastic autoimmune multiorgan syndrome and opens a new challenging investigation avenue for a better understanding of PNP pathogenesis.https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/20805888/pdf/?tool=EBI |
| spellingShingle | Isabelle Schepens Fabienne Jaunin Nadja Begre Ursula Läderach Katrin Marcus Takashi Hashimoto Bertrand Favre Luca Borradori The protease inhibitor alpha-2-macroglobulin-like-1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human. PLoS ONE |
| title | The protease inhibitor alpha-2-macroglobulin-like-1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human. |
| title_full | The protease inhibitor alpha-2-macroglobulin-like-1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human. |
| title_fullStr | The protease inhibitor alpha-2-macroglobulin-like-1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human. |
| title_full_unstemmed | The protease inhibitor alpha-2-macroglobulin-like-1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human. |
| title_short | The protease inhibitor alpha-2-macroglobulin-like-1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human. |
| title_sort | protease inhibitor alpha 2 macroglobulin like 1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human |
| url | https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/20805888/pdf/?tool=EBI |
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