Periplakin (PPL)

Periplakin is a structural protein encoded by the PPL gene in humans, classified as a member of the plakin family[5][2][3]. It is predominantly expressed in keratinocytes and incorporated into the cornified envelope, contributing to the protective barrier function of the epidermis[1][3][5]. The protein contains distinct domains: its N-terminal region interacts with the plasma membrane, the rod domain facilitates complex formation with envoplakin (another plakin family protein), and its C-terminus interacts with intermediate filaments, anchoring desmosomes to the cornified envelope[2][3][1]. Periplakin plays a key role in tissue structural integrity and is implicated as an autoantigen in paraneoplastic pemphigus, an autoimmune blistering disease[3]. It has limited tissue distribution but is essential for epidermal barrier formation and cellular architecture. There are currently no therapeutic agents directed at periplakin.

Other names

190 kDa paraneoplastic pemphigus antigen195 kDa cornified envelope precursor proteinKIAA0568Periplakin

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