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Muscle-Related Plectinopathies
Review

Plectin in Skin Fragility Disorders

1
Department of Dermatology, Faculty of Medicine, Medical Center-University of Freiburg, 79104 Freiburg, Germany
2
MediClin Herzzentrum Lahr/Baden, 77933 Lahr, Germany
*
Author to whom correspondence should be addressed.
Academic Editor: Gerhard Wiche
Cells 2021, 10(10), 2738; https://doi.org/10.3390/cells10102738
Received: 30 July 2021 / Revised: 4 October 2021 / Accepted: 5 October 2021 / Published: 14 October 2021
(This article belongs to the Special Issue Plectin in Health and Disease)
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, PLEC, 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. View Full-Text
Keywords: epidermolysis bullosa; paraneoplastic pemphigus; hemidesmosome; intermediate filaments; plakin; skin blistering; muscular dystrophy; pyloric atresia epidermolysis bullosa; paraneoplastic pemphigus; hemidesmosome; intermediate filaments; plakin; skin blistering; muscular dystrophy; pyloric atresia
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MDPI and ACS Style

Kiritsi, D.; Tsakiris, L.; Schauer, F. Plectin in Skin Fragility Disorders. Cells 2021, 10, 2738. https://doi.org/10.3390/cells10102738

AMA Style

Kiritsi D, Tsakiris L, Schauer F. Plectin in Skin Fragility Disorders. Cells. 2021; 10(10):2738. https://doi.org/10.3390/cells10102738

Chicago/Turabian Style

Kiritsi, Dimitra, Leonidas Tsakiris, and Franziska Schauer. 2021. "Plectin in Skin Fragility Disorders" Cells 10, no. 10: 2738. https://doi.org/10.3390/cells10102738

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