Gene Symbol: Perp
Description: PERP, TP53 apoptosis effector
Alias: 1110017A08Rik, KCP1, KRTCAP1, PIGPC1, THW, p53 apoptosis effector related to PMP-22, KCP-1, keratinocyte-associated protein 1, p53 apoptosis effector related to Pmp22, p53 apoptosis-associated target
Species: mouse
Products:     Perp

Top Publications

  1. Ihrie R, Reczek E, Horner J, Khachatrian L, Sage J, Jacks T, et al. Perp is a mediator of p53-dependent apoptosis in diverse cell types. Curr Biol. 2003;13:1985-90 pubmed
    ..The Perp (p53 apoptosis effector related to PMP-22) gene is highly expressed in cells undergoing p53-dependent apoptosis as ..
  2. Nguyen B, Dusek R, BEAUDRY V, Marinkovich M, Attardi L. Loss of the desmosomal protein perp enhances the phenotypic effects of pemphigus vulgaris autoantibodies. J Invest Dermatol. 2009;129:1710-8 pubmed publisher
    ..Here, we characterize the expression and functional importance of Perp, a newly discovered tetraspan desmosomal protein, in PV...
  3. Ihrie R, Marques M, Nguyen B, Horner J, Papazoglu C, Bronson R, et al. Perp is a p63-regulated gene essential for epithelial integrity. Cell. 2005;120:843-56 pubmed
    ..We show here that Perp, a tetraspan membrane protein originally identified as an apoptosis-associated target of the p53 tumor suppressor, ..
  4. BEAUDRY V, Jiang D, Dusek R, Park E, Knezevich S, Ridd K, et al. Loss of the p53/p63 regulated desmosomal protein Perp promotes tumorigenesis. PLoS Genet. 2010;6:e1001168 pubmed publisher
    ..we investigate the consequences of desmosome loss for carcinogenesis by analyzing conditional knockout mice lacking Perp, a p53/p63 regulated gene that encodes an important component of desmosomes...
  5. Attardi L, Reczek E, Cosmas C, Demicco E, McCurrach M, Lowe S, et al. PERP, an apoptosis-associated target of p53, is a novel member of the PMP-22/gas3 family. Genes Dev. 2000;14:704-18 pubmed
    ..Here, we describe the characterization of a gene identified in this screen, PERP, which is expressed in a p53-dependent manner and at high levels in apoptotic cells compared with G(1)-arrested ..
  6. Marques M, Horner J, Ihrie R, Bronson R, Attardi L. Mice lacking the p53/p63 target gene Perp are resistant to papilloma development. Cancer Res. 2005;65:6551-6 pubmed
    b>Perp is a target of the p53 tumor suppressor involved in the DNA damage-induced apoptosis pathway...
  7. Ihrie R, Bronson R, Attardi L. Adult mice lacking the p53/p63 target gene Perp are not predisposed to spontaneous tumorigenesis but display features of ectodermal dysplasia syndromes. Cell Death Differ. 2006;13:1614-8 pubmed
  8. Hemann M, Zilfou J, Zhao Z, Burgess D, Hannon G, Lowe S. Suppression of tumorigenesis by the p53 target PUMA. Proc Natl Acad Sci U S A. 2004;101:9333-8 pubmed
    ..Additionally, they demonstrate the utility of RNA interference for evaluating putative tumor suppressor genes in vivo. ..
  9. Richardson R, Mitchell K, Hammond N, Mollo M, Kouwenhoven E, Wyatt N, et al. p63 exerts spatio-temporal control of palatal epithelial cell fate to prevent cleft palate. PLoS Genet. 2017;13:e1006828 pubmed publisher

More Information


  1. Fouillade C, Baron Menguy C, Domenga Denier V, Thibault C, Takamiya K, Huganir R, et al. Transcriptome analysis for Notch3 target genes identifies Grip2 as a novel regulator of myogenic response in the cerebrovasculature. Arterioscler Thromb Vasc Biol. 2013;33:76-86 pubmed publisher
    ..Notch3-regulated transcriptome provides potential for modulating myogenic response in the cerebrovasculature. ..
  2. Jheon A, Mostowfi P, Snead M, Ihrie R, Sone E, Pramparo T, et al. PERP regulates enamel formation via effects on cell-cell adhesion and gene expression. J Cell Sci. 2011;124:745-54 pubmed publisher
    Little is known about the role of cell-cell adhesion in the development of mineralized tissues. Here we report that PERP, a tetraspan membrane protein essential for epithelial integrity, regulates enamel formation...
  3. Singaravelu K, Devalaraja Narashimha K, Lastovica B, Padanilam B. PERP, a p53 proapoptotic target, mediates apoptotic cell death in renal ischemia. Am J Physiol Renal Physiol. 2009;296:F847-58 pubmed publisher
    ..elucidate the p53-dependent pathway, we investigated the role of the p53 apoptosis effector related to PMP-22 (PERP), an apoptosis-associated p53 transcriptional target...
  4. Johnson T, Hammond E, Giaccia A, Attardi L. The p53QS transactivation-deficient mutant shows stress-specific apoptotic activity and induces embryonic lethality. Nat Genet. 2005;37:145-52 pubmed
    ..Taken together, these results suggest that p53 has different mechanisms of action depending on specific contextual cues, which may help to clarify the function of p53 in preventing cancer. ..
  5. Reczek E, Flores E, Tsay A, Attardi L, Jacks T. Multiple response elements and differential p53 binding control Perp expression during apoptosis. Mol Cancer Res. 2003;1:1048-57 pubmed
    ..However, the mechanism by which the apoptosis versus arrest decision is made remains to be elucidated. Perp is a proapoptotic target gene of p53 expressed to high levels in apoptotic cells compared with those undergoing ..
  6. Lo Iacono N, Mantero S, Chiarelli A, Garcia E, Mills A, Morasso M, et al. Regulation of Dlx5 and Dlx6 gene expression by p63 is involved in EEC and SHFM congenital limb defects. Development. 2008;135:1377-88 pubmed publisher
    ..ChIP analysis shows that p63 is directly associated with the Dlx5 and Dlx6 promoters. Thus, our data strongly implicate p63 and the Dlx5-Dlx6 locus in a pathway relevant in the aetio-pathogenesis of SHFM...
  7. Jones N, Lynn M, Gaudenz K, Sakai D, Aoto K, Rey J, et al. Prevention of the neurocristopathy Treacher Collins syndrome through inhibition of p53 function. Nat Med. 2008;14:125-33 pubmed publisher
  8. Marques M, Ihrie R, Horner J, Attardi L. The requirement for perp in postnatal viability and epithelial integrity reflects an intrinsic role in stratified epithelia. J Invest Dermatol. 2006;126:69-73 pubmed
    Mice lacking the desmosome protein Perp exhibit blistering in their stratified epithelia and display postnatal lethality...
  9. Ferone G, Mollo M, Thomason H, Antonini D, Zhou H, Ambrosio R, et al. p63 control of desmosome gene expression and adhesion is compromised in AEC syndrome. Hum Mol Genet. 2013;22:531-43 pubmed publisher
    ..Reduced mechanical strength resulting from p63 mutations can be alleviated pharmacologically by increasing desmosome adhesion with possible therapeutic implications...
  10. Neupane S, Sohn W, Rijal G, Lee Y, Lee S, Yamamoto H, et al. Developmental regulations of Perp in mice molar morphogenesis. Cell Tissue Res. 2014;358:109-21 pubmed publisher
    Teraspanin transmembrane protein, Perp (P53 apoptosis effector related to PMP22), which is found in the plasma membrane as a component of the desmosome, is reported to be involved in the morphogenesis of the epithelium and the enamel ..
  11. Abbas H, Maccio D, Coskun S, Jackson J, Hazen A, SILLS T, et al. Mdm2 is required for survival of hematopoietic stem cells/progenitors via dampening of ROS-induced p53 activity. Cell Stem Cell. 2010;7:606-17 pubmed publisher
    ..p16 was also stabilized because of ROS, and its loss increased cell cycling and partially rescued hematopoiesis and survival. Thus, Mdm2 is required to control ROS-induced p53 levels for sustainable hematopoiesis. ..
  12. Wolff S, Talos F, Palacios G, Beyer U, Dobbelstein M, Moll U. The alpha/beta carboxy-terminal domains of p63 are required for skin and limb development. New insights from the Brdm2 mouse which is not a complete p63 knockout but expresses p63 gamma-like proteins. Cell Death Differ. 2009;16:1108-17 pubmed publisher
    ..Thus, Brdm2 mice are p63alpha/beta isoform-specific knockout mice, gaining unexpected new importance. Our studies identify that p63alpha/beta but not p63gamma are absolutely required for proper skin and limb development. ..
  13. Dusek R, Bascom J, Vogel H, Baron S, Borowsky A, Bissell M, et al. Deficiency of the p53/p63 target Perp alters mammary gland homeostasis and promotes cancer. Breast Cancer Res. 2012;14:R65 pubmed
    b>Perp is a transcriptional target of both p53 during DNA damage-induced apoptosis and p63 during stratified epithelial development...
  14. LU J, Izvolsky K, Qian J, Cardoso W. Identification of FGF10 targets in the embryonic lung epithelium during bud morphogenesis. J Biol Chem. 2005;280:4834-41 pubmed
    ..Our approach identifies Fgf10 targets that are common to multiple biological processes and provides insights into potential mechanisms by which Fgf signaling regulates epithelial cell behavior. ..