| Modification Notes |
The Perp protein, also known as p53 (apoptosis) effector related to PMP22, keratinocytes-associated protein 1 (KCP 1), p53-induced protein PIGPC1 or transmembrane protein THW, contains 193 amino acids and has four transmembrane domains, two extracellular domains and three cytoplasmic domains. Northern blot analysis using a multiple tissue array demonstrates that Perp is predominantly expressed in esophagus and trachea tissues. Perp is required for the integrity of the stratified epithelia defined by p63 (a tumor suppressor gene); expression of Perp in these structures is contingent on the presence of p63. The position of Perp downstream of p63 and p53, as well as its essential role in regular desmosome function, suggest that it, like other adhesion proteins, may be a target for mutation in human blistering diseases or cancer. Furthermore, research demonstrates that the downregulation of Perp correlates with meta-static capacity of human melanoma cell lines, indicating that Perp may be a tumor suppressor. |