Spink5-deficient mice mimic Netherton syndrome through degradation of desmoglein 1 by epidermal protease hyperactivity - Test2 - elhamkhani - TriplyDB

Spink5-deficient mice mimic Netherton syndrome through degradation of desmoglein 1 by epidermal protease hyperactivity

Spink5-deficient mice mimic Netherton syndrome through degradation of desmoglein 1 by epidermal protease hyperactivity

http://www.wikidata.org/entity/Q28300081

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Elastase 2 is expressed in human and mouse epidermis and impairs skin barrier function in Netherton syndrome through filaggrin and lipid misprocessing Mutations in CSTA, encoding Cystatin A, underlie exfoliative ichthyosis and reveal a role for this protease inhibitor in cell-cell adhesion Mutations in SERPINB7, encoding a member of the serine protease inhibitor superfamily, cause Nagashima-type palmoplantar keratosis Matriptase initiates activation of epidermal pro-kallikrein and disease onset in a mouse model of Netherton syndrome Protease and protease-activated receptor-2 signaling in the pathogenesis of atopic dermatitis Chymotryptic specificity determinants in the 1.0 A structure of the zinc-inhibited human tissue kallikrein 7 LEKTI fragments specifically inhibit KLK5, KLK7, and KLK14 and control desquamation through a pH-dependent interaction The SPINK gene family and celiac disease susceptibility KLK5 Inactivation Reverses Cutaneous Hallmarks of Netherton Syndrome Filaggrin in the frontline: role in skin barrier function and disease Spink2 modulates apoptotic susceptibility and is a candidate gene in the Rgcs1 QTL that affects retinal ganglion cell death after optic nerve damage 12R-lipoxygenase deficiency disrupts epidermal barrier function Impaired spermatogenesis and fertility in mice carrying a mutation in the Spink2 gene expressed predominantly in testes Identification of lympho-epithelial Kazal-type inhibitor 2 in human skin as a kallikrein-related peptidase 5-specific protease inhibitor Multiple kallikrein (KLK 5, 7, 8, and 10) expression in squamous cell carcinoma of the oral cavity Kallikrein-related peptidase-8 (KLK8) is an active serine protease in human epidermis and sweat and is involved in a skin barrier proteolytic cascade.Mast cells in atopic dermatitis Evolutionary history of tissue kallikreins C/EBPδ gene targets in human keratinocytes.The KLK5 protease suppresses breast cancer by repressing the mevalonate pathway Cervical remodeling during pregnancy and parturition.Filaggrin haploinsufficiency is highly penetrant and is associated with increased severity of eczema: further delineation of the skin phenotype in a prospective epidemiological study of 792 school children.Membrane-anchored serine proteases in vertebrate cell and developmental biology The biology of the desmosome-like junction a versatile anchoring junction and signal transducer in the seminiferous epithelium.Lamellar granule secretion starts before the establishment of tight junction barrier for paracellular tracers in mammalian epidermis Epistasis between serine protease inhibitor Kazal-type 5 (SPINK5) and thymic stromal lymphopoietin (TSLP) genes contributes to childhood asthma Ex-vivo gene therapy restores LEKTI activity and corrects the architecture of Netherton syndrome-derived skin grafts Epidermal barrier formation and recovery in skin disorders.Kallikrein-5 promotes cleavage of desmoglein-1 and loss of cell-cell cohesion in oral squamous cell carcinoma.Hair follicular expression and function of group X secreted phospholipase A2 in mouse skin.Matriptase: potent proteolysis on the cell surface.TLR2 expression is increased in rosacea and stimulates enhanced serine protease production by keratinocytes.PAR2 absence completely rescues inflammation and ichthyosis caused by altered CAP1/Prss8 expression in mouse skin.Light microscopic hair abnormalities in children: retrospective review of 119 cases in a 10-year period.Delineation of matriptase protein expression by enzymatic gene trapping suggests diverging roles in barrier function, hair formation, and squamous cell carcinogenesis Structure, function, and regulation of desmosomes.Inhibition of transcription factor specificity protein 1 alters the gene expression profile of keratinocytes leading to upregulation of kallikrein-related peptidases and thymic stromal lymphopoietin.Autosomal recessive ichthyosis with hypotrichosis caused by a mutation in ST14, encoding type II transmembrane serine protease matriptase.Human involucrin promoter mediates repression-resistant and compartment-specific LEKTI expression Deconstructing the skin: cytoarchitectural determinants of epidermal morphogenesis

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P2860

Spink5-deficient mice mimic Netherton syndrome through degradation of desmoglein 1 by epidermal protease hyperactivity

http://www.wikidata.org/entity/Q28300081

description

2005 nî lūn-bûn

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2005 թուականի Յունուարին հրատարակուած գիտական յօդուած

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2005 թվականի հունվարին հրատարակված գիտական հոդված

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2005年の論文

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2005年論文

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2005年論文

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2005年论文

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name

Spink5-deficient mice mimic Ne ...... idermal protease hyperactivity

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Spink5-deficient mice mimic Ne ...... idermal protease hyperactivity

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Spink5-deficient mice mimic Ne ...... idermal protease hyperactivity

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Spink5-deficient mice mimic Ne ...... idermal protease hyperactivity

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Spink5-deficient mice mimic Ne ...... idermal protease hyperactivity

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Spink5-deficient mice mimic Ne ...... idermal protease hyperactivity

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Spink5-deficient mice mimic Ne ...... idermal protease hyperactivity

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Spink5-deficient mice mimic Ne ...... idermal protease hyperactivity

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Spink5-deficient mice mimic Ne ...... idermal protease hyperactivity

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Nature Genetics

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Spink5-deficient mice mimic Ne ...... idermal protease hyperactivity

@en

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Arnoud Sonnenberg

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Chrystelle Bonnart

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Céline Deraison

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Giovanna Zambruno

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Maaike Kreft

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Mari Kishibe

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Pascal Descargues

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Peter Elias

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Yann Barrandon

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P2860

LEKTI, a novel 15-domain type of human serine proteinase inhibitor

Purification, molecular cloning, and expression of a human stratum corneum trypsin-like serine protease with possible function in desquamation

Characterization and purification of human corneodesmosin, an epidermal basic glycoprotein associated with corneocyte-specific modified desmosomes

LEKTI proteolytic processing in human primary keratinocytes, tissue distribution and defective expression in Netherton syndrome

Epidermal detachment, desmosomal dissociation, and destabilization of corneodesmosin in Spink5-/- mice

Klf4 is a transcription factor required for establishing the barrier function of the skin

Mutations in SPINK5, encoding a serine protease inhibitor, cause Netherton syndrome

Inhibition of serine proteinases plasmin, trypsin, subtilisin A, cathepsin G, and elastase by LEKTI: a kinetic analysis

Netherton syndrome: disease expression and spectrum of SPINK5 mutations in 21 families

Elevated stratum corneum hydrolytic activity in Netherton syndrome suggests an inhibitory regulation of desquamation by SPINK5-derived peptides

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P304

56-65

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scholarly article

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10.1038/NG1493

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P433

1

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P478

37

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Alain Hovnanian

Akemi Ishida-Yamamoto

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2005-01-01T00:00:00Z

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8110312

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15619623

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