Perp

Summary

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

Top Publications

  1. ncbi Perp is a p63-regulated gene essential for epithelial integrity
    Rebecca A Ihrie
    Division of Radiation and Cancer Biology, Stanford University School of Medicine, Stanford, California 94305, USA
    Cell 120:843-56. 2005
  2. ncbi Adult mice lacking the p53/p63 target gene Perp are not predisposed to spontaneous tumorigenesis but display features of ectodermal dysplasia syndromes
    R A Ihrie
    Cell Death Differ 13:1614-8. 2006
  3. pmc Loss of the desmosomal protein perp enhances the phenotypic effects of pemphigus vulgaris autoantibodies
    Bichchau Nguyen
    Division of Radiation and Cancer Biology, Department of Radiation Oncology, Stanford University School of Medicine, Stanford, California 94305 5152, USA
    J Invest Dermatol 129:1710-8. 2009
  4. ncbi Mice lacking the p53/p63 target gene Perp are resistant to papilloma development
    Michelle R Marques
    Department of Radiation Oncology, Division of Radiation and Cancer Biology, Stanford University School of Medicine, Stanford, California 94305, USA
    Cancer Res 65:6551-6. 2005
  5. ncbi Perp is a mediator of p53-dependent apoptosis in diverse cell types
    Rebecca A Ihrie
    Division of Radiation and Cancer Biology, Department of Radiation Oncology, Stanford University School of Medicine, Stanford, CA 94305, USA
    Curr Biol 13:1985-90. 2003
  6. pmc PERP, an apoptosis-associated target of p53, is a novel member of the PMP-22/gas3 family
    L D Attardi
    Department of Biology and Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139 USA
    Genes Dev 14:704-18. 2000
  7. pmc Suppression of tumorigenesis by the p53 target PUMA
    Michael T Hemann
    Cold Spring Harbor Laboratory, 1 Bungtown Road, Cold Spring Harbor, NY 11724, USA
    Proc Natl Acad Sci U S A 101:9333-8. 2004
  8. pmc Deficiency of the p53/p63 target Perp alters mammary gland homeostasis and promotes cancer
    Rachel L Dusek
    Division of Radiation and Cancer Biology, Department of Radiation Oncology, Stanford University School of Medicine, Center for Clinical Sciences Research Room 1255, 269 Campus Drive, Stanford, CA 94305, USA
    Breast Cancer Res 14:R65. 2012
  9. pmc p63 control of desmosome gene expression and adhesion is compromised in AEC syndrome
    Giustina Ferone
    Fondazione IRCCS SDN, Napoli, Italy
    Hum Mol Genet 22:531-43. 2013
  10. pmc The requirement for perp in postnatal viability and epithelial integrity reflects an intrinsic role in stratified epithelia
    Michelle R Marques
    Department of Radiation Oncology, Division of Radiation and Cancer Biology, Stanford University School of Medicine, Stanford, California 94305, USA
    J Invest Dermatol 126:69-73. 2006

Detail Information

Publications20

  1. ncbi Perp is a p63-regulated gene essential for epithelial integrity
    Rebecca A Ihrie
    Division of Radiation and Cancer Biology, Stanford University School of Medicine, Stanford, California 94305, USA
    Cell 120:843-56. 2005
    ..We show here that Perp, a tetraspan membrane protein originally identified as an apoptosis-associated target of the p53 tumor suppressor, ..
  2. ncbi Adult mice lacking the p53/p63 target gene Perp are not predisposed to spontaneous tumorigenesis but display features of ectodermal dysplasia syndromes
    R A Ihrie
    Cell Death Differ 13:1614-8. 2006
  3. pmc Loss of the desmosomal protein perp enhances the phenotypic effects of pemphigus vulgaris autoantibodies
    Bichchau Nguyen
    Division of Radiation and Cancer Biology, Department of Radiation Oncology, Stanford University School of Medicine, Stanford, California 94305 5152, USA
    J Invest Dermatol 129:1710-8. 2009
    ..Here, we characterize the expression and functional importance of Perp, a newly discovered tetraspan desmosomal protein, in PV...
  4. ncbi Mice lacking the p53/p63 target gene Perp are resistant to papilloma development
    Michelle R Marques
    Department of Radiation Oncology, Division of Radiation and Cancer Biology, Stanford University School of Medicine, Stanford, California 94305, USA
    Cancer Res 65:6551-6. 2005
    b>Perp is a target of the p53 tumor suppressor involved in the DNA damage-induced apoptosis pathway...
  5. ncbi Perp is a mediator of p53-dependent apoptosis in diverse cell types
    Rebecca A Ihrie
    Division of Radiation and Cancer Biology, Department of Radiation Oncology, Stanford University School of Medicine, Stanford, CA 94305, USA
    Curr Biol 13:1985-90. 2003
    ..The Perp (p53 apoptosis effector related to PMP-22) gene is highly expressed in cells undergoing p53-dependent apoptosis as ..
  6. pmc PERP, an apoptosis-associated target of p53, is a novel member of the PMP-22/gas3 family
    L D Attardi
    Department of Biology and Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139 USA
    Genes Dev 14:704-18. 2000
    ..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 ..
  7. pmc Suppression of tumorigenesis by the p53 target PUMA
    Michael T Hemann
    Cold Spring Harbor Laboratory, 1 Bungtown Road, Cold Spring Harbor, NY 11724, USA
    Proc Natl Acad Sci U S A 101:9333-8. 2004
    ..Additionally, they demonstrate the utility of RNA interference for evaluating putative tumor suppressor genes in vivo...
  8. pmc Deficiency of the p53/p63 target Perp alters mammary gland homeostasis and promotes cancer
    Rachel L Dusek
    Division of Radiation and Cancer Biology, Department of Radiation Oncology, Stanford University School of Medicine, Center for Clinical Sciences Research Room 1255, 269 Campus Drive, Stanford, CA 94305, USA
    Breast Cancer Res 14:R65. 2012
    b>Perp is a transcriptional target of both p53 during DNA damage-induced apoptosis and p63 during stratified epithelial development...
  9. pmc p63 control of desmosome gene expression and adhesion is compromised in AEC syndrome
    Giustina Ferone
    Fondazione IRCCS SDN, Napoli, Italy
    Hum Mol Genet 22:531-43. 2013
    ..Reduced mechanical strength resulting from p63 mutations can be alleviated pharmacologically by increasing desmosome adhesion with possible therapeutic implications...
  10. pmc The requirement for perp in postnatal viability and epithelial integrity reflects an intrinsic role in stratified epithelia
    Michelle R Marques
    Department of Radiation Oncology, Division of Radiation and Cancer Biology, Stanford University School of Medicine, Stanford, California 94305, USA
    J Invest Dermatol 126:69-73. 2006
    Mice lacking the desmosome protein Perp exhibit blistering in their stratified epithelia and display postnatal lethality...
  11. ncbi Multiple response elements and differential p53 binding control Perp expression during apoptosis
    Elizabeth E Reczek
    Department of Biology and Center for Cancer Research, Massachusetts Institute of Technology, 77 Massachusetts Avenue, Cambridge, MA 02139, USA
    Mol Cancer Res 1:1048-57. 2003
    ..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 ..
  12. pmc PERP regulates enamel formation via effects on cell-cell adhesion and gene expression
    Andrew H Jheon
    Department of Orofacial Sciences and Program in Craniofacial and Mesenchymal Biology, University of California, San Francisco, CA 94143, USA
    J Cell Sci 124:745-54. 2011
    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...
  13. pmc Mdm2 is required for survival of hematopoietic stem cells/progenitors via dampening of ROS-induced p53 activity
    Hussein A Abbas
    Program in Genes and Development of The Graduate School of Biomedical Sciences, University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
    Cell Stem Cell 7:606-17. 2010
    ..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...
  14. pmc Loss of the p53/p63 regulated desmosomal protein Perp promotes tumorigenesis
    VERONICA G BEAUDRY
    Department of Radiation Oncology, Division of Radiation and Cancer Biology, Stanford University School of Medicine, Stanford, California, USA
    PLoS Genet 6:e1001168. 2010
    ..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...
  15. pmc 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
    S Wolff
    Department of Molecular Oncology, University of Gottingen, Gottingen, Germany
    Cell Death Differ 16:1108-17. 2009
    ..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...
  16. ncbi Identification of FGF10 targets in the embryonic lung epithelium during bud morphogenesis
    JINING LU
    Pulmonary Center, Boston University School of Medicine, Boston, Massachusetts 02118, USA
    J Biol Chem 280:4834-41. 2005
    ..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...
  17. doi PERP, a p53 proapoptotic target, mediates apoptotic cell death in renal ischemia
    Kurinji Singaravelu
    Dept of Cellular and Integrative Physiology, Nebraska Medical Center, Omaha, NE 68198 5850, USA
    Am J Physiol Renal Physiol 296:F847-58. 2009
    ..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...
  18. doi Regulation of Dlx5 and Dlx6 gene expression by p63 is involved in EEC and SHFM congenital limb defects
    Nadia Lo Iacono
    Dulbecco Telethon Institute, Molecular Biotechnology Center, University of Torino, Via Nizza 52, Torino, 10126, Italy
    Development 135:1377-88. 2008
    ..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...
  19. ncbi The p53QS transactivation-deficient mutant shows stress-specific apoptotic activity and induces embryonic lethality
    Thomas M Johnson
    Division of Radiation and Cancer Biology, Department of Radiation Oncology, Stanford University School of Medicine, California 94305, USA
    Nat Genet 37:145-52. 2005
    ..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...
  20. pmc Prevention of the neurocristopathy Treacher Collins syndrome through inhibition of p53 function
    Natalie C Jones
    Stowers Institute for Medical Research, 1000 East 50th Street, Kansas City, Missouri 64110, USA
    Nat Med 14:125-33. 2008
    ....