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iRHOM2: A Regulator of Palmoplantar Biology, Inflammation, and Viral Susceptibility

The palmoplantar epidermis is a specialized area of the skin that undergoes high levels of mechanical stress. The palmoplantar keratinization and esophageal cancer syndrome, tylosis with esophageal cancer, is linked to mutations in RHBDF2 encoding the proteolytically inactive rhomboid protein, iRhom...

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Detalles Bibliográficos
Autores principales: Chao-Chu, Jennifer, Murtough, Stephen, Zaman, Najwa, Pennington, Daniel J., Blaydon, Diana C., Kelsell, David P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Authors. Published by Elsevier, Inc. on behalf of the Society for Investigative Dermatology. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7568177/
https://www.ncbi.nlm.nih.gov/pubmed/33080304
http://dx.doi.org/10.1016/j.jid.2020.09.010
Descripción
Sumario:The palmoplantar epidermis is a specialized area of the skin that undergoes high levels of mechanical stress. The palmoplantar keratinization and esophageal cancer syndrome, tylosis with esophageal cancer, is linked to mutations in RHBDF2 encoding the proteolytically inactive rhomboid protein, iRhom2. Subsequently, iRhom2 was found to affect palmoplantar thickening to modulate the stress keratin response and to mediate context-dependent stress pathways by p63. iRhom2 is also a direct regulator of the sheddase, ADAM17, and the antiviral adaptor protein, stimulator of IFN genes. In this perspective, the pleiotropic functions of iRhom2 are discussed with respect to the skin, inflammation, and the antiviral response.