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Epiplakin is a large cytoskeletal linker protein encoded by the EPPK1 gene, notable for its unique structure composed entirely of plakin repeat domains and for containing the entire coding region within a single exon[1][3][4]. It belongs to the plakin family, whose members connect intermediate filaments (such as keratins) and support stress resistance in tissues[2][4]. Epiplakin is primarily found in epithelial tissues, including the skin and cornea. It binds to keratin intermediate filaments, influencing their organization and stability in response to stress and injury, and plays a protective role by regulating keratin filament reorganization and protecting epithelial tissue architecture during wound healing and cellular stress[2][3][7]. Epiplakin has been identified as an autoantigen in paraneoplastic pemphigus, a rare autoimmune blistering skin disorder, suggesting it may serve as a biomarker in some disease contexts[1][3]. No known drugs currently target epiplakin, and it is not regarded as a direct therapeutic target such as a receptor or enzyme[3][7][8].
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