title: Identifying a hyperkeratosis signature in autosomal recessive congenital ichthyosis: Mdm2 inhibition prevents hyperkeratosis in a rat ARCI model.
authors.affiliation: Livingstone Skin Research Centre, Institute of Child Health, UCL, London, UK; Immunobiology and Dermatology, Institute of Child Health, UCL, London, UK.
authors.forename: Gehad
authors.initials: G
authors.lastname: Youssef
authors.affiliation: Immunobiology and Dermatology, Institute of Child Health, UCL, London, UK.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Ono
authors.affiliation: Livingstone Skin Research Centre, Institute of Child Health, UCL, London, UK; Immunobiology and Dermatology, Institute of Child Health, UCL, London, UK.
authors.forename: Stuart J
authors.initials: SJ
authors.lastname: Brown
authors.affiliation: Livingstone Skin Research Centre, Institute of Child Health, UCL, London, UK; Clinical and Molecular Genetics Unit, Institute of Child Health, UCL, London, UK.
authors.forename: Veronica A
authors.initials: VA
authors.lastname: Kinsler
authors.affiliation: Histopathology, Great Ormond Street Hospital, London, UK.
authors.forename: Neil J
authors.initials: NJ
authors.lastname: Sebire
authors.affiliation: Livingstone Skin Research Centre, Institute of Child Health, UCL, London, UK; Immunobiology and Dermatology, Institute of Child Health, UCL, London, UK.
authors.forename: John I
authors.initials: JI
authors.lastname: Harper
authors.affiliation: Livingstone Skin Research Centre, Institute of Child Health, UCL, London, UK; Immunobiology and Dermatology, Institute of Child Health, UCL, London, UK. Electronic address: r.oshaughnessy@ucl.ac.uk.
authors.forename: Ryan F L
authors.initials: RFL
authors.lastname: O'Shaughnessy
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D005808:Genes, Recessive; D006801:Humans; D017490:Ichthyosis, Lamellar / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D007093:Imidazoles / Q000494:pharmacology*; D007642:Keratosis / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism; D009924:Organ Culture Techniques; D010879:Piperazines / Q000494:pharmacology*; D051736:Proto-Oncogene Proteins c-mdm2 / Q000037:antagonists & inhibitors* / Q000235:genetics; D051381:Rats; D012867:Skin / Q000473:pathology*
chemical_list: D007093:Imidazoles; D010879:Piperazines; C482205:nutlin 3; C497208:Mdm2 protein, rat; D051736:Proto-Oncogene Proteins c-mdm2