The Desmosomal Plaque Proteins of the Plakophilin Family
Three related proteins of the plakophilin family (PKP1_3) have been identified as junctional proteins that are essential for the formation and stabilization of desmosomal cell contacts. Failure of PKP expression can have fatal effects on desmosomal adhesion, leading to abnormal tissue and organ deve...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2010-01-01
|
| Series: | Dermatology Research and Practice |
| Online Access: | http://dx.doi.org/10.1155/2010/101452 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832545489127473152 |
|---|---|
| author | Steffen Neuber Mario Mühmer Denise Wratten Peter J. Koch Roland Moll Ansgar Schmidt |
| author_facet | Steffen Neuber Mario Mühmer Denise Wratten Peter J. Koch Roland Moll Ansgar Schmidt |
| author_sort | Steffen Neuber |
| collection | DOAJ |
| description | Three related proteins of the plakophilin family (PKP1_3) have been identified as junctional proteins that are essential for the formation and stabilization of desmosomal cell contacts. Failure of PKP expression can have fatal effects on desmosomal adhesion, leading to abnormal tissue and organ development. Thus, loss of functional PKP 1 in humans leads to ectodermal dysplasia/skin fragility (EDSF) syndrome, a genodermatosis with severe blistering of the epidermis as well as abnormal keratinocytes differentiation. Mutations in the human PKP 2 gene have been linked to severe heart abnormalities that lead to arrhythmogenic right ventricular cardiomyopathy (ARVC). In the past few years it has been shown that junctional adhesion is not the only function of PKPs. These proteins have been implicated in cell signaling, organization of the cytoskeleton, and control of protein biosynthesis under specific cellular circumstances. Clearly, PKPs are more than just cell adhesion proteins. In this paper we will give an overview of our current knowledge on the very distinct roles of plakophilins in the cell. |
| format | Article |
| id | doaj-art-2077f4edca84465094c39a561f7cc28d |
| institution | Kabale University |
| issn | 1687-6105 1687-6113 |
| language | English |
| publishDate | 2010-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Dermatology Research and Practice |
| spelling | doaj-art-2077f4edca84465094c39a561f7cc28d2025-02-03T07:25:33ZengWileyDermatology Research and Practice1687-61051687-61132010-01-01201010.1155/2010/101452101452The Desmosomal Plaque Proteins of the Plakophilin FamilySteffen Neuber0Mario Mühmer1Denise Wratten2Peter J. Koch3Roland Moll4Ansgar Schmidt5Institute of Pathology, Philipps University of Marburg, Baldingerstraße, 35033 Marburg, GermanyInstitute of Pathology, Philipps University of Marburg, Baldingerstraße, 35033 Marburg, GermanyInstitute of Pathology, Philipps University of Marburg, Baldingerstraße, 35033 Marburg, GermanyDepartments of Dermatology, Cell & Developmental Biology, University of Colorado Medical School, Aurora, CO 80045, USAInstitute of Pathology, Philipps University of Marburg, Baldingerstraße, 35033 Marburg, GermanyInstitute of Pathology, Philipps University of Marburg, Baldingerstraße, 35033 Marburg, GermanyThree related proteins of the plakophilin family (PKP1_3) have been identified as junctional proteins that are essential for the formation and stabilization of desmosomal cell contacts. Failure of PKP expression can have fatal effects on desmosomal adhesion, leading to abnormal tissue and organ development. Thus, loss of functional PKP 1 in humans leads to ectodermal dysplasia/skin fragility (EDSF) syndrome, a genodermatosis with severe blistering of the epidermis as well as abnormal keratinocytes differentiation. Mutations in the human PKP 2 gene have been linked to severe heart abnormalities that lead to arrhythmogenic right ventricular cardiomyopathy (ARVC). In the past few years it has been shown that junctional adhesion is not the only function of PKPs. These proteins have been implicated in cell signaling, organization of the cytoskeleton, and control of protein biosynthesis under specific cellular circumstances. Clearly, PKPs are more than just cell adhesion proteins. In this paper we will give an overview of our current knowledge on the very distinct roles of plakophilins in the cell.http://dx.doi.org/10.1155/2010/101452 |
| spellingShingle | Steffen Neuber Mario Mühmer Denise Wratten Peter J. Koch Roland Moll Ansgar Schmidt The Desmosomal Plaque Proteins of the Plakophilin Family Dermatology Research and Practice |
| title | The Desmosomal Plaque Proteins of the Plakophilin Family |
| title_full | The Desmosomal Plaque Proteins of the Plakophilin Family |
| title_fullStr | The Desmosomal Plaque Proteins of the Plakophilin Family |
| title_full_unstemmed | The Desmosomal Plaque Proteins of the Plakophilin Family |
| title_short | The Desmosomal Plaque Proteins of the Plakophilin Family |
| title_sort | desmosomal plaque proteins of the plakophilin family |
| url | http://dx.doi.org/10.1155/2010/101452 |
| work_keys_str_mv | AT steffenneuber thedesmosomalplaqueproteinsoftheplakophilinfamily AT mariomuhmer thedesmosomalplaqueproteinsoftheplakophilinfamily AT denisewratten thedesmosomalplaqueproteinsoftheplakophilinfamily AT peterjkoch thedesmosomalplaqueproteinsoftheplakophilinfamily AT rolandmoll thedesmosomalplaqueproteinsoftheplakophilinfamily AT ansgarschmidt thedesmosomalplaqueproteinsoftheplakophilinfamily AT steffenneuber desmosomalplaqueproteinsoftheplakophilinfamily AT mariomuhmer desmosomalplaqueproteinsoftheplakophilinfamily AT denisewratten desmosomalplaqueproteinsoftheplakophilinfamily AT peterjkoch desmosomalplaqueproteinsoftheplakophilinfamily AT rolandmoll desmosomalplaqueproteinsoftheplakophilinfamily AT ansgarschmidt desmosomalplaqueproteinsoftheplakophilinfamily |