title: Plakins, a versatile family of cytolinkers: roles in skin integrity and in human diseases.
abstract: The plakin family consists of giant proteins involved in the cross-linking and organization of the cytoskeleton and adhesion complexes. They further modulate several fundamental biological processes, such as cell adhesion, migration, and polarization or signaling pathways. Inherited and acquired defects of plakins in humans and in animal models potentially lead to dramatic manifestations in the skin, striated muscles, and/or nervous system. These observations unequivocally demonstrate the key role of plakins in the maintenance of tissue integrity. Here we review the characteristics of the mammalian plakin members BPAG1 (bullous pemphigoid antigen 1), desmoplakin, plectin, envoplakin, epiplakin, MACF1 (microtubule-actin cross-linking factor 1), and periplakin, highlighting their role in skin homeostasis and diseases.
authors.affiliation: Departments of Dermatology and Clinical Research, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland; Graduate School for Cellular and Biomedical Sciences, University of Bern, Bern, Switzerland.
authors.forename: Jamal-Eddine
authors.initials: JE
authors.lastname: Bouameur
authors.affiliation: Departments of Dermatology and Clinical Research, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland. Electronic address: Bertrand.Favre@insel.ch.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Favre
authors.affiliation: Departments of Dermatology and Clinical Research, Inselspital, Bern University Hospital, and University of Bern, Bern, Switzerland.
authors.forename: Luca
authors.initials: L
authors.lastname: Borradori
mesh_terms: D000818:Animals; D001327:Autoimmune Diseases / Q000235:genetics; D002448:Cell Adhesion; D002465:Cell Movement; D003599:Cytoskeleton / Q000378:metabolism; D004195:Disease Models, Animal; D005786:Gene Expression Regulation; D006706:Homeostasis; D006801:Humans; D051379:Mice; D009154:Mutation; D009369:Neoplasms / Q000235:genetics; D010802:Phylogeny; D051179:Plakins / Q000235:genetics* / Q000502:physiology*; D015398:Signal Transduction; D012867:Skin / Q000378:metabolism; D012879:Skin Physiological Phenomena
chemical_list: D051179:Plakins