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The Desmosomal Plaque Proteins of the Plakophilin Family
Joint Authors
Moll, Roland
Wratten, Denise
Koch, Peter J.
Mühmer, Mario
Neuber, Steffen
Schmidt, Ansgar
Source
Dermatology Research and Practice
Issue
Vol. 2010, Issue 2010 (31 Dec. 2010), pp.1-11, 11 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2010-04-21
Country of Publication
Egypt
No. of Pages
11
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Neuber, Steffen& Mühmer, Mario& Wratten, Denise& Koch, Peter J.& Moll, Roland& Schmidt, Ansgar. 2010. The Desmosomal Plaque Proteins of the Plakophilin Family. Dermatology Research and Practice،Vol. 2010, no. 2010, pp.1-11.
https://search.emarefa.net/detail/BIM-446374
Modern Language Association (MLA)
Neuber, Steffen…[et al.]. The Desmosomal Plaque Proteins of the Plakophilin Family. Dermatology Research and Practice No. 2010 (2010), pp.1-11.
https://search.emarefa.net/detail/BIM-446374
American Medical Association (AMA)
Neuber, Steffen& Mühmer, Mario& Wratten, Denise& Koch, Peter J.& Moll, Roland& Schmidt, Ansgar. The Desmosomal Plaque Proteins of the Plakophilin Family. Dermatology Research and Practice. 2010. Vol. 2010, no. 2010, pp.1-11.
https://search.emarefa.net/detail/BIM-446374
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-446374