Plectin in Skin Fragility Disorders
Abstract
:1. Introduction
2. Plectin in Hereditary Skin Fragility Disorders
Subtype | Plectin Isotype Affected | Mode of Inheritance | Organs Affected | Mouse Model |
---|---|---|---|---|
EBS-PA | 1 | AR | Skin, mucosa, pylorus, urinary tract (muscle) | [45], die 2–3 days after birth |
EBS-MD | 1 | AR | Skin, mucosa, muscle, enamel, laryngotracheal and gastrointestinal | [45], died 2–3 days after birth |
Limb girdle muscular dystrophy type 2Q | 1f | AR | Muscle | [46] |
EBS-MD with myasthenic symptoms | 1 | AR | Muscle | NA |
EBS Ogna | rod domain | AD | Skin | [45] |
EBS-plectin 1a | 1a | AR | Skin | [17] |
3. Plectin in Autoimmune Skin Fragility Disorders
4. The Role of Plectin in Fibroblast Organization
5. Treatment Option for Patients with EBS-MD
6. Future Perspectives
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Acknowledgments
Conflicts of Interest
References
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Kiritsi, D.; Tsakiris, L.; Schauer, F. Plectin in Skin Fragility Disorders. Cells 2021, 10, 2738. https://doi.org/10.3390/cells10102738
Kiritsi D, Tsakiris L, Schauer F. Plectin in Skin Fragility Disorders. Cells. 2021; 10(10):2738. https://doi.org/10.3390/cells10102738
Chicago/Turabian StyleKiritsi, Dimitra, Leonidas Tsakiris, and Franziska Schauer. 2021. "Plectin in Skin Fragility Disorders" Cells 10, no. 10: 2738. https://doi.org/10.3390/cells10102738