Plectin in Skin Fragility Disorders
Plectin is a multi-faceted, 500 kDa-large protein, which due to its expression in different isoforms and distinct organs acts diversely as a cytoskeletal crosslinker and signaling scaffold. It functions as a mediator of keratinocyte mechanical stability in the skin, primarily through linking interme...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-10-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/10/10/2738 |
| _version_ | 1797514974307811328 |
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| author | Dimitra Kiritsi Leonidas Tsakiris Franziska Schauer |
| author_facet | Dimitra Kiritsi Leonidas Tsakiris Franziska Schauer |
| author_sort | Dimitra Kiritsi |
| collection | DOAJ |
| description | Plectin is a multi-faceted, 500 kDa-large protein, which due to its expression in different isoforms and distinct organs acts diversely as a cytoskeletal crosslinker and signaling scaffold. It functions as a mediator of keratinocyte mechanical stability in the skin, primarily through linking intermediate filaments to hemidesmosomes. Skin fragility may occur through the presence of mutations in the gene encoding for plectin, <i>PLEC</i>, or through the presence of autoantibodies against the molecule. Below, we review the cutaneous manifestations of plectinopathies as well as their systemic involvement in specific disease subtypes. We summarize the known roles of plectin in keratinocytes and fibroblasts and provide an outlook on future perspectives for plectin-associated skin disorders. |
| first_indexed | 2024-03-10T06:39:07Z |
| format | Article |
| id | doaj.art-03365dc952fb4566a44481be8b8c31b1 |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-10T06:39:07Z |
| publishDate | 2021-10-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-03365dc952fb4566a44481be8b8c31b12023-11-22T17:48:24ZengMDPI AGCells2073-44092021-10-011010273810.3390/cells10102738Plectin in Skin Fragility DisordersDimitra Kiritsi0Leonidas Tsakiris1Franziska Schauer2Department of Dermatology, Faculty of Medicine, Medical Center-University of Freiburg, 79104 Freiburg, GermanyMediClin Herzzentrum Lahr/Baden, 77933 Lahr, GermanyDepartment of Dermatology, Faculty of Medicine, Medical Center-University of Freiburg, 79104 Freiburg, GermanyPlectin is a multi-faceted, 500 kDa-large protein, which due to its expression in different isoforms and distinct organs acts diversely as a cytoskeletal crosslinker and signaling scaffold. It functions as a mediator of keratinocyte mechanical stability in the skin, primarily through linking intermediate filaments to hemidesmosomes. Skin fragility may occur through the presence of mutations in the gene encoding for plectin, <i>PLEC</i>, or through the presence of autoantibodies against the molecule. Below, we review the cutaneous manifestations of plectinopathies as well as their systemic involvement in specific disease subtypes. We summarize the known roles of plectin in keratinocytes and fibroblasts and provide an outlook on future perspectives for plectin-associated skin disorders.https://www.mdpi.com/2073-4409/10/10/2738epidermolysis bullosaparaneoplastic pemphigushemidesmosomeintermediate filamentsplakinskin blistering |
| spellingShingle | Dimitra Kiritsi Leonidas Tsakiris Franziska Schauer Plectin in Skin Fragility Disorders Cells epidermolysis bullosa paraneoplastic pemphigus hemidesmosome intermediate filaments plakin skin blistering |
| title | Plectin in Skin Fragility Disorders |
| title_full | Plectin in Skin Fragility Disorders |
| title_fullStr | Plectin in Skin Fragility Disorders |
| title_full_unstemmed | Plectin in Skin Fragility Disorders |
| title_short | Plectin in Skin Fragility Disorders |
| title_sort | plectin in skin fragility disorders |
| topic | epidermolysis bullosa paraneoplastic pemphigus hemidesmosome intermediate filaments plakin skin blistering |
| url | https://www.mdpi.com/2073-4409/10/10/2738 |
| work_keys_str_mv | AT dimitrakiritsi plectininskinfragilitydisorders AT leonidastsakiris plectininskinfragilitydisorders AT franziskaschauer plectininskinfragilitydisorders |