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    KLK5 and KLK7 Ablation Fully Rescues Lethality of Netherton Syndrome-Like Phenotype

    January 2017 in “ PLoS Genetics ”
    Petr Kašpárek, Zuzana Ileninova, Olga Zbodakova … Radislav Sedláček
    Studysummary This study found that simultaneously inhibiting both KLK5 and KLK7 proteases completely rescued skin barrier defects in a mouse model of Netherton syndrome, suggesting both should be therapeutic targets.
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    Research cited in this study 8

    1. Protease Activity, Localization, and Inhibition in the Human Hair Follicle International Journal of Cosmetic Science · 2013
    2. Corneodesmosin Gene Ablation Induces Lethal Skin-Barrier Disruption and Hair-Follicle Degeneration Related to Desmosome Dysfunction Journal of Cell Science · 2009
    3. Distribution of Caspase-14 in Epidermis and Hair Follicles Is Evolutionarily Conserved Among Mammals ˜The œanatomical record. Part A, Discoveries in molecular, cellular, and evolutionary biology/Anatomical record. Part A, Discoveries in molecular, cellular, and evolutionary biology · 2005
    4. Spink5-Deficient Mice Mimic Netherton Syndrome Through Degradation of Desmoglein 1 by Epidermal Protease Hyperactivity Nature Genetics · 2004
    5. Mice Lacking Desmocollin 1 Show Epidermal Fragility Accompanied by Barrier Defects and Abnormal Differentiation The Journal of Cell Biology · 2001
    6. Lanceolate Hair (Lah): A Recessive Mouse Mutation with Alopecia and Abnormal Hair ˜The œjournal of investigative dermatology/Journal of investigative dermatology · 1996
    7. Netherton's Syndrome: A Syndrome of Elevated IgE and Characteristic Skin and Hair Findings ˆThe ‰journal of allergy and clinical immunology/Journal of allergy and clinical immunology/˜The œjournal of allergy and clinical immunology · 1995
    8. Pathogenesis in Trichorrhexis Invaginata (Bamboo Hair) Journal of Investigative Dermatology · 1984