Proteases in Pemphigoid Diseases
Pemphigoid diseases are a subgroup of autoimmune skin diseases characterized by widespread tense blisters. Standard of care typically involves immunosuppressive treatments, which may be insufficient and are often associated with significant adverse events. As such, a deeper understanding of the path...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2019-06-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fimmu.2019.01454/full |
| _version_ | 1819112039617921024 |
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| author | Sho Hiroyasu Sho Hiroyasu Sho Hiroyasu Christopher T. Turner Christopher T. Turner Christopher T. Turner Katlyn C. Richardson Katlyn C. Richardson Katlyn C. Richardson David J. Granville David J. Granville David J. Granville |
| author_facet | Sho Hiroyasu Sho Hiroyasu Sho Hiroyasu Christopher T. Turner Christopher T. Turner Christopher T. Turner Katlyn C. Richardson Katlyn C. Richardson Katlyn C. Richardson David J. Granville David J. Granville David J. Granville |
| author_sort | Sho Hiroyasu |
| collection | DOAJ |
| description | Pemphigoid diseases are a subgroup of autoimmune skin diseases characterized by widespread tense blisters. Standard of care typically involves immunosuppressive treatments, which may be insufficient and are often associated with significant adverse events. As such, a deeper understanding of the pathomechanism(s) of pemphigoid diseases is necessary in order to identify improved therapeutic approaches. A major initiator of pemphigoid diseases is the accumulation of autoantibodies against proteins at the dermal-epidermal junction (DEJ), followed by protease activation at the lesion. The contribution of proteases to pemphigoid disease pathogenesis has been investigated using a combination of in vitro and in vivo models. These studies suggest proteolytic degradation of anchoring proteins proximal to the DEJ is crucial for dermal-epidermal separation and blister formation. In addition, proteases can also augment inflammation, expose autoantigenic cryptic epitopes, and/or provoke autoantigen spreading, which are all important in pemphigoid disease pathology. The present review summarizes and critically evaluates the current understanding with respect to the role of proteases in pemphigoid diseases. |
| first_indexed | 2024-12-22T04:07:10Z |
| format | Article |
| id | doaj.art-6880641c273342278bc5aed347307a4a |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-12-22T04:07:10Z |
| publishDate | 2019-06-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-6880641c273342278bc5aed347307a4a2022-12-21T18:39:37ZengFrontiers Media S.A.Frontiers in Immunology1664-32242019-06-011010.3389/fimmu.2019.01454463154Proteases in Pemphigoid DiseasesSho Hiroyasu0Sho Hiroyasu1Sho Hiroyasu2Christopher T. Turner3Christopher T. Turner4Christopher T. Turner5Katlyn C. Richardson6Katlyn C. Richardson7Katlyn C. Richardson8David J. Granville9David J. Granville10David J. Granville11International Collaboration On Repair Discoveries (ICORD), Vancouver Coastal Health Research Institute (VCHRI), Vancouver, BC, CanadaDepartment of Pathology and Laboratory Medicine, University of British Columbia (UBC), Vancouver, BC, CanadaBC Professional Firefighters' Burn and Wound Healing Group, Vancouver Coastal Health Research Institute (VCHRI), University of British Columbia (UBC), Vancouver, BC, CanadaInternational Collaboration On Repair Discoveries (ICORD), Vancouver Coastal Health Research Institute (VCHRI), Vancouver, BC, CanadaDepartment of Pathology and Laboratory Medicine, University of British Columbia (UBC), Vancouver, BC, CanadaBC Professional Firefighters' Burn and Wound Healing Group, Vancouver Coastal Health Research Institute (VCHRI), University of British Columbia (UBC), Vancouver, BC, CanadaInternational Collaboration On Repair Discoveries (ICORD), Vancouver Coastal Health Research Institute (VCHRI), Vancouver, BC, CanadaDepartment of Pathology and Laboratory Medicine, University of British Columbia (UBC), Vancouver, BC, CanadaBC Professional Firefighters' Burn and Wound Healing Group, Vancouver Coastal Health Research Institute (VCHRI), University of British Columbia (UBC), Vancouver, BC, CanadaInternational Collaboration On Repair Discoveries (ICORD), Vancouver Coastal Health Research Institute (VCHRI), Vancouver, BC, CanadaDepartment of Pathology and Laboratory Medicine, University of British Columbia (UBC), Vancouver, BC, CanadaBC Professional Firefighters' Burn and Wound Healing Group, Vancouver Coastal Health Research Institute (VCHRI), University of British Columbia (UBC), Vancouver, BC, CanadaPemphigoid diseases are a subgroup of autoimmune skin diseases characterized by widespread tense blisters. Standard of care typically involves immunosuppressive treatments, which may be insufficient and are often associated with significant adverse events. As such, a deeper understanding of the pathomechanism(s) of pemphigoid diseases is necessary in order to identify improved therapeutic approaches. A major initiator of pemphigoid diseases is the accumulation of autoantibodies against proteins at the dermal-epidermal junction (DEJ), followed by protease activation at the lesion. The contribution of proteases to pemphigoid disease pathogenesis has been investigated using a combination of in vitro and in vivo models. These studies suggest proteolytic degradation of anchoring proteins proximal to the DEJ is crucial for dermal-epidermal separation and blister formation. In addition, proteases can also augment inflammation, expose autoantigenic cryptic epitopes, and/or provoke autoantigen spreading, which are all important in pemphigoid disease pathology. The present review summarizes and critically evaluates the current understanding with respect to the role of proteases in pemphigoid diseases.https://www.frontiersin.org/article/10.3389/fimmu.2019.01454/fullpemphigoid diseasesproteasesbullous pemphigoidepidermolysis bullosa acquisitamucous membrane pemphigoidelastase |
| spellingShingle | Sho Hiroyasu Sho Hiroyasu Sho Hiroyasu Christopher T. Turner Christopher T. Turner Christopher T. Turner Katlyn C. Richardson Katlyn C. Richardson Katlyn C. Richardson David J. Granville David J. Granville David J. Granville Proteases in Pemphigoid Diseases Frontiers in Immunology pemphigoid diseases proteases bullous pemphigoid epidermolysis bullosa acquisita mucous membrane pemphigoid elastase |
| title | Proteases in Pemphigoid Diseases |
| title_full | Proteases in Pemphigoid Diseases |
| title_fullStr | Proteases in Pemphigoid Diseases |
| title_full_unstemmed | Proteases in Pemphigoid Diseases |
| title_short | Proteases in Pemphigoid Diseases |
| title_sort | proteases in pemphigoid diseases |
| topic | pemphigoid diseases proteases bullous pemphigoid epidermolysis bullosa acquisita mucous membrane pemphigoid elastase |
| url | https://www.frontiersin.org/article/10.3389/fimmu.2019.01454/full |
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