Matriptase drives early-onset intestinal failure in a mouse model of congenital tufting enteropathy

Claudin-7 gene knockout causes destruction of intestinal structure and animal death in mice

(2019) Chang Xu et al.   WORLD JOURNAL OF GASTROENTEROLOGY

Functions of EpCAM in physiological processes and diseases (Review)

(2018) Li Huang et al.   INTERNATIONAL JOURNAL OF MOLECULAR MEDICINE

Hepatocyte growth factor activator inhibitors (HAI-1 and HAI-2): Emerging key players in epithelial integrity and cancer

(2018) Hiroaki Kataoka et al.   PATHOLOGY INTERNATIONAL

Loss of HAI-2 in mice with decreased prostasin activity leads to an early-onset intestinal failure resembling congenital tufting enteropathy

(2018) Roman Szabo et al.   PLoS One

SPINT2 (HAI-2) missense variants identified in congenital sodium diarrhea/tufting enteropathy affect the ability of HAI-2 to inhibit prostasin but not matriptase

(2018) Lasse Holt-Danborg et al.   HUMAN MOLECULAR GENETICS

Matriptase-mediated cleavage of EpCAM destabilizes claudins and dysregulates intestinal epithelial homeostasis

(2017) Chuan-Jin Wu et al.   JOURNAL OF CLINICAL INVESTIGATION

A Nonsense Variant in the ST14 Gene in Akhal-Teke Horses with Naked Foal Syndrome

(2017) Anina Bauer et al.   G3-Genes Genomes Genetics

En Route to New Therapeutic Options for Iron Overload Diseases: Matriptase-2 as a Target for Kunitz-Type Inhibitors

(2016) Anna-Madeleine Beckmann et al.   CHEMBIOCHEM

Delineation of proteolytic and non-proteolytic functions of the membrane-anchored serine protease prostasin

(2016) Roman Szabo et al.   DEVELOPMENT

Altered Prostasin (CAP1/Prss8) Expression Favors Inflammation and Tissue Remodeling in DSS-induced Colitis

(2016) Anna Keppner et al.   INFLAMMATORY BOWEL DISEASES

Novel p.Glu519Gln missense mutation in ST14 in a patient with ichthyosis, follicular atrophoderma and hypotrichosis and review of the literature

(2016) Iria Neri et al.   JOURNAL OF DERMATOLOGICAL SCIENCE

Intestinal deletion ofClaudin-7enhances paracellular organic solute flux and initiates colonic inflammation in mice

(2015) Hiroo Tanaka et al.   GUT

Distinct Developmental Functions of Prostasin (CAP1/PRSS8) Zymogen and Activated Prostasin

(2015) Stine Friis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Colon-Specific Deletion of Epithelial Sodium Channel Causes Sodium Loss and Aldosterone Resistance

(2014) S. Malsure et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Functional Analysis of a Missense Mutation in the Serine Protease Inhibitor SPINT2 Associated with Congenital Sodium Diarrhea

(2014) Nicolas Faller et al.   PLoS One

Regulation of Feto-Maternal Barrier by Matriptase- and PAR-2-Mediated Signaling Is Required for Placental Morphogenesis and Mouse Embryonic Survival

(2014) Roman Szabo et al.   PLoS Genetics

Functional consequences of EpCam mutation in mice and men

(2013) James L. Mueller et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY

Hepatocyte growth factor activator inhibitor type 2 (HAI-2) modulates hepcidin expression by inhibiting the cell surface protease matriptase-2

(2013) Eva Maurer et al.   BIOCHEMICAL JOURNAL

EpCAM Controls Actomyosin Contractility and Cell Adhesion by Direct Inhibition of PKC

(2013) Nadim Maghzal et al.   DEVELOPMENTAL CELL

Genetic characterization of congenital tufting enteropathy: epcam associated phenotype and involvement of SPINT2 in the syndromic form

(2013) Julie Salomon et al.   HUMAN GENETICS

A Matriptase-Prostasin Reciprocal Zymogen Activation Complex with Unique Features

(2013) Stine Friis et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Prostasin Is Required for Matriptase Activation in Intestinal Epithelial Cells to Regulate Closure of the Paracellular Pathway

(2013) Marguerite S. Buzza et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutations of the Serine Protease CAP1/Prss8 Lead to Reduced Embryonic Viability, Skin Defects, and Decreased ENaC Activity

(2012) Simona Frateschi et al.   AMERICAN JOURNAL OF PATHOLOGY

EpCAM contributes to formation of functional tight junction in the intestinal epithelium by recruiting claudin proteins

(2012) Zili Lei et al.   DEVELOPMENTAL BIOLOGY

mTrop1/Epcam Knockout Mice Develop Congenital Tufting Enteropathy through Dysregulation of Intestinal E-cadherin/β-catenin

(2012) Emanuela Guerra et al.   PLoS One

Reduced Prostasin (CAP1/PRSS8) Activity Eliminates HAI-1 and HAI-2 Deficiency–Associated Developmental Defects by Preventing Matriptase Activation

(2012) Roman Szabo et al.   PLoS Genetics

Inflammation and Disruption of the Mucosal Architecture in Claudin-7–Deficient Mice

(2011) Lei Ding et al.   GASTROENTEROLOGY

Suppression of Tumorigenicity-14, encoding matriptase, is a critical suppressor of colitis and colitis-associated colon carcinogenesis

(2011) P Kosa et al.   ONCOGENE

Local Protease Signaling Contributes to Neural Tube Closure in the Mouse Embryo

(2010) Eric Camerer et al.   DEVELOPMENTAL CELL

Mutations in SPINT2 Cause a Syndromic Form of Congenital Sodium Diarrhea

(2009) Peter Heinz-Erian et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Epithelial Integrity Is Maintained by a Matriptase-Dependent Proteolytic Pathway

(2009) Karin List et al.   AMERICAN JOURNAL OF PATHOLOGY

Autosomal recessive ichthyosis with hypotrichosis syndrome: further delineation of the phenotype

(2009) L Avrahami et al.   CLINICAL GENETICS

Regulation of cell surface protease matriptase by HAI2 is essential for placental development, neural tube closure and embryonic survival in mice

(2009) R. Szabo et al.   DEVELOPMENT

ENaC-mediated alveolar fluid clearance and lung fluid balance depend on the channel-activating protease 1

(2009) Carole Planès et al.   EMBO Molecular Medicine

Nuclear signalling by tumour-associated antigen EpCAM

(2009) Dorothea Maetzel et al.   NATURE CELL BIOLOGY

Identification of EpCAM as the Gene for Congenital Tufting Enteropathy

(2008) Mamata Sivagnanam et al.   GASTROENTEROLOGY

Potent Inhibition and Global Co-localization Implicate the Transmembrane Kunitz-type Serine Protease Inhibitor Hepatocyte Growth Factor Activator Inhibitor-2 in the Regulation of Epithelial Matriptase Activity

(2008) Roman Szabo et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ichthyosis, Follicular Atrophoderma, and Hypotrichosis Caused by Mutations in ST14 Is Associated with Impaired Profilaggrin Processing

(2008) Thomas Alef et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Structure of an Fab–Protease Complex Reveals a Highly Specific Non-canonical Mechanism of Inhibition

(2008) Christopher J. Farady et al.   JOURNAL OF MOLECULAR BIOLOGY

Zymogen activation, inhibition, and ectodomain shedding of matriptase

(2007) Chen-Yong Lin   Frontiers in Bioscience-Landmark