Gilles Courtois

Summary

Country: France

Publications

  1. ncbi request reprint The NF-kappaB signaling pathway in human genetic diseases
    Gilles Courtois
    INSERM U697, Pavillon Bazin, Hopital Saint Louis, 1, avenue Claude Vellefaux, 75010, Paris, France
    Cell Mol Life Sci 62:1682-91. 2005
  2. ncbi request reprint NF-kappaB-related genetic diseases
    G Courtois
    INSERM U697, Pavillon Bazin, Hopital Saint Louis, Paris, France
    Cell Death Differ 13:843-51. 2006
  3. doi request reprint Tumor suppressor CYLD: negative regulation of NF-kappaB signaling and more
    G Courtois
    INSERM U697, Pavillon Bazin, Hopital Saint Louis, 1 avenue Claude Vellefaux, Paris, France
    Cell Mol Life Sci 65:1123-32. 2008
  4. doi request reprint Inhibition of IkappaB kinase subunit 2 in cutaneous T-cell lymphoma down-regulates nuclear factor-kappaB constitutive activation, induces cell death, and potentiates the apoptotic response to antineoplastic chemotherapeutic agents
    Aurore Sors
    Unité Institut National de la Sante et de la Recherche Medicale U697 and Department of Dermatology 1, Hopital Saint Louis, AP HP, Universite Paris 7, Paris, France
    Clin Cancer Res 14:901-11. 2008
  5. doi request reprint Inducible expression and pathophysiologic functions of T-plastin in cutaneous T-cell lymphoma
    Elodie Bégué
    Centre de Recherche sur la Peau, Universite Paris Diderot, Hopital Saint Louis, Paris, France
    Blood 120:143-54. 2012
  6. ncbi request reprint A point mutation in NEMO associated with anhidrotic ectodermal dysplasia with immunodeficiency pathology results in destabilization of the oligomer and reduces lipopolysaccharide- and tumor necrosis factor-mediated NF-kappa B activation
    Emilie Vinolo
    Unité de Régulation Enzymatique des Activités Cellulaires, CNRS URA 2185, Paris, France
    J Biol Chem 281:6334-48. 2006
  7. ncbi request reprint Identification of TRAF6-dependent NEMO polyubiquitination sites through analysis of a new NEMO mutation causing incontinentia pigmenti
    Hélène Sebban-Benin
    INSERM U697, Pavilion Bazin, Hopital Saint Louis, Paris, France
    Hum Mol Genet 16:2805-15. 2007
  8. ncbi request reprint NF-kappaB and inflammation in genetic disease
    Hélène Sebban
    INSERM U697, Pavillon Bazin, Hopital Saint Louis, 1, avenue Claude Vellefaux, 75010 Paris, France
    Biochem Pharmacol 72:1153-60. 2006
  9. pmc A hypermorphic IkappaBalpha mutation is associated with autosomal dominant anhidrotic ectodermal dysplasia and T cell immunodeficiency
    Gilles Courtois
    Unité de Biologie Moléculaire de l Expression Génique, Centre National de la Recherche Scientifique URA 2582, Institut Pasteur, Paris, France
    J Clin Invest 112:1108-15. 2003
  10. ncbi request reprint Osteopetrosis, lymphedema, anhidrotic ectodermal dysplasia, and immunodeficiency in a boy and incontinentia pigmenti in his mother
    Sophie Dupuis-Girod
    Unité d Immunologie et d Hé matologie pédiatriques, Hopital Necker Enfants Malades, Paris, France
    Pediatrics 109:e97. 2002

Collaborators

Detail Information

Publications32

  1. ncbi request reprint The NF-kappaB signaling pathway in human genetic diseases
    Gilles Courtois
    INSERM U697, Pavillon Bazin, Hopital Saint Louis, 1, avenue Claude Vellefaux, 75010, Paris, France
    Cell Mol Life Sci 62:1682-91. 2005
    ..In this review, a summary of the pathophysiological consequences of impaired NF-kappaB activation in humans is provided with respect to the functions of the molecules which are mutated...
  2. ncbi request reprint NF-kappaB-related genetic diseases
    G Courtois
    INSERM U697, Pavillon Bazin, Hopital Saint Louis, Paris, France
    Cell Death Differ 13:843-51. 2006
    ..In addition, the identified mutations will help understanding at the molecular level how NF-kappaB is activated in response to cell stimulation...
  3. doi request reprint Tumor suppressor CYLD: negative regulation of NF-kappaB signaling and more
    G Courtois
    INSERM U697, Pavillon Bazin, Hopital Saint Louis, 1 avenue Claude Vellefaux, Paris, France
    Cell Mol Life Sci 65:1123-32. 2008
    ....
  4. doi request reprint Inhibition of IkappaB kinase subunit 2 in cutaneous T-cell lymphoma down-regulates nuclear factor-kappaB constitutive activation, induces cell death, and potentiates the apoptotic response to antineoplastic chemotherapeutic agents
    Aurore Sors
    Unité Institut National de la Sante et de la Recherche Medicale U697 and Department of Dermatology 1, Hopital Saint Louis, AP HP, Universite Paris 7, Paris, France
    Clin Cancer Res 14:901-11. 2008
    ..We investigated in vitro the effects on CTCL survival and chemoresistance of a specific inhibition of IkappaB kinase subunit 2 (IKK2)...
  5. doi request reprint Inducible expression and pathophysiologic functions of T-plastin in cutaneous T-cell lymphoma
    Elodie Bégué
    Centre de Recherche sur la Peau, Universite Paris Diderot, Hopital Saint Louis, Paris, France
    Blood 120:143-54. 2012
    ..In conclusion, T-plastin is a marker restricted to malignant lymphocytes from SS patients and plays a role for cell survival and migration. This opens new strategies for the treatment of SS advanced stages...
  6. ncbi request reprint A point mutation in NEMO associated with anhidrotic ectodermal dysplasia with immunodeficiency pathology results in destabilization of the oligomer and reduces lipopolysaccharide- and tumor necrosis factor-mediated NF-kappa B activation
    Emilie Vinolo
    Unité de Régulation Enzymatique des Activités Cellulaires, CNRS URA 2185, Paris, France
    J Biol Chem 281:6334-48. 2006
    ..Altogether, our findings provide understanding as to how a single point mutation in NEMO leads to the observed EDA-ID phenotype in relation to the NEMO-dependent mechanism of IKK activation...
  7. ncbi request reprint Identification of TRAF6-dependent NEMO polyubiquitination sites through analysis of a new NEMO mutation causing incontinentia pigmenti
    Hélène Sebban-Benin
    INSERM U697, Pavilion Bazin, Hopital Saint Louis, Paris, France
    Hum Mol Genet 16:2805-15. 2007
    ..These findings indicate that post-translational modification of NEMO through K63-linked polyubiquitination is a key event in IKK activation and that perturbation of this step may cause human pathophysiology...
  8. ncbi request reprint NF-kappaB and inflammation in genetic disease
    Hélène Sebban
    INSERM U697, Pavillon Bazin, Hopital Saint Louis, 1, avenue Claude Vellefaux, 75010 Paris, France
    Biochem Pharmacol 72:1153-60. 2006
    ....
  9. pmc A hypermorphic IkappaBalpha mutation is associated with autosomal dominant anhidrotic ectodermal dysplasia and T cell immunodeficiency
    Gilles Courtois
    Unité de Biologie Moléculaire de l Expression Génique, Centre National de la Recherche Scientifique URA 2582, Institut Pasteur, Paris, France
    J Clin Invest 112:1108-15. 2003
    ..Our report highlights both the diversity of genotypes associated with EDA-ID and the diversity of immunologic phenotypes associated with mutations in different components of the NF-kappaB signaling pathway...
  10. ncbi request reprint Osteopetrosis, lymphedema, anhidrotic ectodermal dysplasia, and immunodeficiency in a boy and incontinentia pigmenti in his mother
    Sophie Dupuis-Girod
    Unité d Immunologie et d Hé matologie pédiatriques, Hopital Necker Enfants Malades, Paris, France
    Pediatrics 109:e97. 2002
    ..In contrast, loss-of-function mutations and hypomorphic mutations may cause IP in females...
  11. ncbi request reprint Posttranslational modifications of NEMO and its partners in NF-kappaB signaling
    Hélène Sebban
    INSERM U697, Hopital Saint Louis, Paris 75010, France
    Trends Cell Biol 16:569-77. 2006
    ....
  12. doi request reprint IKK regulation and human genetics
    Gilles Courtois
    INSERM U781, Hopital Necker Enfants Malades, Paris, France
    Curr Top Microbiol Immunol 349:73-95. 2011
    ....
  13. doi request reprint "Without Ub I am nothing": NEMO as a multifunctional player in ubiquitin-mediated control of NF-kappaB activation
    Jérémie Gautheron
    INSERM U781, Tour Lavoisier, Hôpital Necker Enfants Malades and Université Paris Descartes, 149, rue de Sevres, 75015, Paris, France
    Cell Mol Life Sci 67:3101-13. 2010
    ....
  14. ncbi request reprint Down-regulating constitutive activation of the NF-kappaB canonical pathway overcomes the resistance of cutaneous T-cell lymphoma to apoptosis
    Aurore Sors
    INSERM U697, Institut de Recherche sur la Peau, Pavillon Bazin, Hopital Saint Louis, 1 avenue Claude Vellefaux, 75475 Paris Cedex 10, France
    Blood 107:2354-63. 2006
    ....
  15. ncbi request reprint NF-kappaB in skin homeostasis
    Gilles Courtois
    INSERM U697, Hopital Saint Louis, Institut de Recherche sur la Peau, 1 avenue Claude Vellefaux, 75010 Paris, France
    Exp Dermatol 14:781-2. 2005
  16. ncbi request reprint Complementation cloning of NEMO, a component of the IkappaB kinase complex essential for NF-kappaB activation
    S Yamaoka
    Unité de Biologie Moléculaire de l Expression Génique, URA 1773 CNRS, Institut Pasteur, Paris, France
    Cell 93:1231-40. 1998
    ..3E2, in which the protein is also absent, allowing us to demonstrate that this factor is required not only for Tax but also for LPS, PMA, and IL-1 stimulation of NF-kappaB activity...
  17. ncbi request reprint Genomic rearrangement in NEMO impairs NF-kappaB activation and is a cause of incontinentia pigmenti. The International Incontinentia Pigmenti (IP) Consortium
    A Smahi
    Department of Genetics, Unité de Recherches sur les Handicaps Génétiques de l Enfant INSERMU 393, Hopital Necker Enfants Malades, Paris, France
    Nature 405:466-72. 2000
    ..Here we show that most cases of IP are due to mutations of this locus and that a new genomic rearrangement accounts for 80% of new mutations. As a consequence, NF-kappaB activation is defective in IP cells...
  18. ncbi request reprint X-linked anhidrotic ectodermal dysplasia with immunodeficiency is caused by impaired NF-kappaB signaling
    R Doffinger
    Laboratoire de Genetique Humaine des Maladies Infectieuses, Faculte de Medecine Necker Enfants Malades, Paris, France
    Nat Genet 27:277-85. 2001
    ..We thus report for the first time that impaired but not abolished NF-kappaB signaling in humans results in two related syndromes that associate specific developmental and immunological defects...
  19. ncbi request reprint Mutations in the NF-kappaB signaling pathway: implications for human disease
    G Courtois
    INSERM U697, Hopital Saint Louis, Paris, France
    Oncogene 25:6831-43. 2006
    ..In addition, human genetic diseases are briefly described wherein mutations affect protein modifiers or transducers of NF-kappaB signaling or disrupt NF-kappaB-binding sites in promoters/enhancers...
  20. ncbi request reprint Autosomal dominant anhidrotic ectodermal dysplasias at the EDARADD locus
    E Bal
    INSERM U781 et Département de Génétique, Hopital Necker Enfants Malades, Paris, France
    Hum Mutat 28:703-9. 2007
    ..Our results confirm that NF-kB activation is impaired in EDA and support the role of EDARADD DD as a downstream effector of EDAR signaling...
  21. ncbi request reprint The tumour suppressor CYLD negatively regulates NF-kappaB signalling by deubiquitination
    Andrew Kovalenko
    Department of Biological Chemistry, The Weizmann Institute of Science, 76100 Rehovot, Israel
    Nature 424:801-5. 2003
    ..Truncations of CYLD found in cylindromatosis result in reduced enzymatic activity, indicating a link between impaired deubiquitination of CYLD substrates and human pathophysiology...
  22. ncbi request reprint Post-induction, stimulus-specific regulation of tumor necrosis factor mRNA expression
    Alla V Tsytsykova
    CBR Institute for Biomedical Research, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Biol Chem 282:11629-38. 2007
    ....
  23. ncbi request reprint The NF-kappaB signalling pathway in human diseases: from incontinentia pigmenti to ectodermal dysplasias and immune-deficiency syndromes
    Asma Smahi
    Département de Génétique et Unité de Recherches sur les Handicaps Génétiques de l Enfant INSERM UR 393, Hopital Necker, 149 rue de Sevres, 75743 Paris Cedex 15, France
    Hum Mol Genet 11:2371-5. 2002
    ..Unravelling the molecular bases of other forms of EDA not associated with mutations in NEMO will possibly implicate other components of the NF-kappaB signalling pathway...
  24. ncbi request reprint [Human pathologies associated with NF-kappaB defects]
    Gilles Courtois
    Unité de Biologie Moléculaire de l Expression Génique, URA 2582 CNRS, Institut Pasteur, 25, rue du Dr Roux, 75724 Paris Cedex 15, France
    J Soc Biol 198:93-6. 2004
    ..This gene encodes a protein which interacts with NEMO and exhibits deubiquitinase activity, therefore strengthening the recent hypothesis of the role of non-degradation-linked ubiquitination in NF-kappaB activation...
  25. ncbi request reprint Molecular analysis of the genetic defect in a large cohort of IP patients and identification of novel NEMO mutations interfering with NF-kappaB activation
    Francesca Fusco
    Institute of Genetics and Biophysics, Adriano Buzzati Traverso CR, Naples, Italy
    Hum Mol Genet 13:1763-73. 2004
    ..Indeed, 64% of our patients have extremely skewed X-inactivation pattern (>/=80 : 20). Overall IP pathogenesis thus depends on a combination of X-inactivation and protein domain that recruit upstream factors and activate NF-kappaB...
  26. ncbi request reprint NEMO trimerizes through its coiled-coil C-terminal domain
    Fabrice Agou
    Unité de Régulation Enzymatique des Activités Cellulaires, Paris Cedex 15, France
    J Biol Chem 277:17464-75. 2002
    ..We propose a model in which the activation of the IKK complex occurs through the trimerization of NEMO upon binding to a not yet identified upstream activator...
  27. ncbi request reprint TNF-mediated inflammatory skin disease in mice with epidermis-specific deletion of IKK2
    Manolis Pasparakis
    Institute for Genetics, University of Cologne, Weyertal 121, D 50931 Cologne, Germany
    Nature 417:861-6. 2002
    ..Our results suggest that the critical function of IKK2-mediated NF-kappa B activity in epidermal keratinocytes is to regulate mechanisms that maintain the immune homeostasis of the skin...
  28. ncbi request reprint The trimerization domain of NEMO is composed of the interacting C-terminal CC2 and LZ coiled-coil subdomains
    Fabrice Agou
    Unité de Régulation Enzymatique des Activités Cellulaires, CNRS URA 2185, Institut Pasteur, 25 rue du Dr Roux, 75724 Paris Cedex 15, France
    J Biol Chem 279:27861-9. 2004
    ..This model is discussed in relation to the mechanism of activation of the IKK complex by upstream activators...
  29. ncbi request reprint Yersinia outer protein P suppresses TGF-beta-activated kinase-1 activity to impair innate immune signaling in Yersinia enterocolitica-infected cells
    Rudolf Haase
    Max von Pettenkofer Institute for Hygiene and Medical Microbiology, Munich, Germany
    J Immunol 175:8209-17. 2005
    ....
  30. ncbi request reprint Two carboxyl-terminal activation regions of Epstein-Barr virus latent membrane protein 1 activate NF-kappaB through distinct signaling pathways in fibroblast cell lines
    Naohito Saito
    Department of Molecular Virology, Graduate School of Medicine, Tokyo Medical and Dental University, Yushima 1 5 45, Bunkyo ku, Tokyo 113 8519, Japan
    J Biol Chem 278:46565-75. 2003
    ..Our results thus help clarify how two functional domains of LMP1 persistently activate NF-kappaB through distinct signaling pathways...
  31. ncbi request reprint Mature T cells depend on signaling through the IKK complex
    Marc Schmidt-Supprian
    Center for Blood Research, Harvard Medical School, 200 Longwood Avenue, Boston, MA 02115, USA
    Immunity 19:377-89. 2003
    ..Thus, IKK-induced NF-kappaB activation, mediated by either IKK1 or IKK2, is essential for the generation and survival of mature T cells, and IKK2 has an additional role in regulatory and memory T cell development...
  32. ncbi request reprint Inhibition of NF-kappa B activation by peptides targeting NF-kappa B essential modulator (nemo) oligomerization
    Fabrice Agou
    Unité de Régulation Enzymatique des Activités Cellulaires, CNRS URA 2185, 75724 Paris, France
    J Biol Chem 279:54248-57. 2004
    ..Our results provide the "proof of concept" for a new and promising strategy for the inhibition of NF-kappa B pathway activation through targeting the oligomerization state of the NEMO protein...