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Matriptase

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https://www.readbyqxmd.com/read/28491880/iron-refractory-iron-deficiency-anemia-in-dizygotic-twins-due-to-a-novel-tmprss6-gene-mutation-in-addition-to-polymorphisms-associated-with-high-susceptibility-to-develop-ferropenic-anemia
#1
Joana Pinto, Gustavo Nobre de Jesus, Mónica Palma Anselmo, Lúcia Gonçalves, Daniela Brás, João Madeira Lopes, João Meneses, Rui Victorino, Paula Faustino
Iron refractory iron deficiency anemia (IRIDA) is an autosomal recessive ferropenic anemia. Its hypochromic microcytic pattern is associated with low transferrin saturation, normal-high ferritin, and inappropriately high hepcidin level. This entity is caused by mutants of the TMPRSS6 gene that encodes the protein matriptase II, which influences hepcidin expression, an iron metabolism counterregulatory protein. We report two 29-year-old dizygotic female twins with ferropenic, hypochromic microcytic anemia with 20 years of evolution, refractory to oral iron therapy...
April 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28490634/inflammatory-cytokines-down-regulate-the-barrier-protective-prostasin-matriptase-proteolytic-cascade-early-in-experimental-colitis
#2
Marguerite S Buzza, Tierra A Johnson, Gregory D Conway, Erik W Martin, Subhradip Mukhopadhyay, Terez Shea-Donohue, Toni M Antalis
Compromised gastrointestinal barrier function is strongly associated with the progressive and destructive pathologies of the two main forms of IBD, Ulcerative Colitis (UC) and Crohns disease (CD). Matriptase is a membrane-anchored serine protease encoded by Suppression of Tumorigenicity-14 (ST14) gene, which is critical for epithelial barrier development and homeostasis. Matriptase barrier protective activity is linked with the glycosyl-phosphatidyl-inositol (GPI)-anchored serine protease prostasin, which is a co-factor for matriptase zymogen activation...
May 10, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28464560/matriptase-induces-metalloproteinase-dependent-aggrecanolysis-in-vitro-and-in-vivo-multiple-mechanisms-promote-cartilage-damage-in-osteoarthritis
#3
David J Wilkinson, Angela Habgood, Heather K Lamb, Paul Thompson, Alastair R Hawkins, Antoine Désilets, Richard Leduc, Torsten Steinmetzer, Maya Hammami, Melody S Lee, Charles S Craik, Sharon Watson, Hua Lin, Jennifer M Milner, Andrew D Rowan
Objectives To assess the ability of the type II transmembrane serine proteinase matriptase to promote aggrecan loss from cartilage of osteoarthritis (OA) patients, and whether matriptase inhibition could prevent aggrecan loss and cartilage damage in experimental OA. Methods Aggrecan release was assessed from human OA cartilage explants and human stem cell-derived cartilage discs, whilst cartilage conditioned media were used for western blotting. Gene expression was analysed by real-time PCR. Murine OA was induced by surgical destabilisation of the medial meniscus, and matriptase inhibitors delivered via osmotic mini-pump or intra-articular injection...
May 2, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28454318/expression-and-prognostic-value-of-matriptase-in-ovarian-serous-adenocarcinoma
#4
Mei Ji, Shunshuang Li, Ya Xie, Zhao Zhao, Weizhong Chang, Yue Li, Xinghan Cheng, Zhuo Wang
Previous studies have demonstrated that matriptase is involved in degradation of the extracellular matrix and angiogenesis, and is overexpressed in certain forms of epithelial cancer. The present study aimed to examine matriptase expression in ovarian serous adenocarcinoma, and to investigate its association with clinicopathological characteristics and patient prognosis. Matriptase expression was analyzed in 80 ovarian serous adenocarcinoma and 12 normal ovarian tissue samples by immunohistochemistry. All data were analyzed to evaluate the association between matriptase expression and clinicopathological parameters and overall survival...
March 2017: Oncology Letters
https://www.readbyqxmd.com/read/28454094/proteolytic-cleavages-in-the-extracellular-domain-of-receptor-tyrosine-kinases-by-membrane-associated-serine-proteases
#5
Li-Mei Chen, Karl X Chai
The epithelial extracellular membrane-associated serine proteases matriptase, hepsin, and prostasin are proteolytic modifying enzymes of the extracellular domain (ECD) of the epidermal growth factor receptor (EGFR). Matriptase also cleaves the ECD of the vascular endothelial growth factor receptor 2 (VEGFR2) and the angiopoietin receptor Tie2. In this study we tested the hypothesis that these serine proteases may cleave the ECD of additional receptor tyrosine kinases (RTKs). We co-expressed the proteases in an epithelial cell line with Her2, Her3, Her4, insulin receptor (INSR), insulin-like growth factor I receptor (IGF-1R), the platelet-derived growth factor receptors (PDGFRs) α and β, or nerve growth factor receptor A (TrkA)...
April 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/28447549/a-child-with-severe-iron-deficiency-anemia-and-a-complex-tmprss6-genotype
#6
Anna Paola Capra, Elisa Ferro, Laura Cannavò, Maria Angela La Rosa, Giuseppina Zirilli
OBJECTIVES: We report a case of a 7-year-old girl with severe hypochromic microcytic anemia, who was unresponsive to classical iron supplements. We suspected IRIDA, iron-refractory iron-deficiency anemia, a genetic iron metabolism disorder, caused by TMPRSS6 variations. TMPRSS6 encodes matriptase-2, a negative regulator of hepcidin, and its pathological variants are related to normal to high levels of hepcidin. We analyzed the TMPRSS6 gene and we improved clinical management of the patient, selecting the appropriate supplementation therapy...
April 27, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28393458/increased-filaggrin-metabolizing-enzyme-activity-in-atopic-skin-a-pilot-study-using-a-canine-model-of-atopic-dermatitis
#7
Natalia Fanton, Domenico Santoro, Luisa Cornegliani, Rosanna Marsella
BACKGROUND: Filaggrin (FLG) and its metabolites are essential for skin barrier function and hydration of the stratum corneum. Alteration of the FLG metabolism could be the basis for an abnormal skin barrier in allergic dogs. OBJECTIVES: To investigate the expression and distribution of calpain-1, caspase-14, furin and matriptase, four enzymes involved in FLG metabolism, in the skin of atopic and healthy beagles. METHODS: Skin biopsies were collected from four healthy and four atopic beagles before and after allergen exposure...
April 9, 2017: Veterinary Dermatology
https://www.readbyqxmd.com/read/28371047/hai-2-stabilizes-inhibits-and-regulates-sea-cleavage-dependent-secretory-transport-of-matriptase
#8
Annika W Nonboe, Oliver Krigslund, Christoffer Soendergaard, Signe Skovbjerg, Stine Friis, Martin N Andersen, Vincent Ellis, Makiko Kawaguchi, Hiroaki Kataoka, Thomas H Bugge, Lotte K Vogel
It has recently been shown that hepatocyte growth factor activator inhibitor-2 (HAI-2) is able to suppress carcinogenesis induced by overexpression of matriptase, as well as cause regression of individual established tumors in a mouse model system. However, the role of HAI-2 is poorly understood. In this study, we describe 3 mutations in the binding loop of the HAI-2 Kunitz domain 1 (K42N, C47F and R48L) that cause a delay in the SEA domain cleavage of matriptase, leading to accumulation of non-SEA domain cleaved matriptase in the endoplasmic reticulum (ER)...
March 28, 2017: Traffic
https://www.readbyqxmd.com/read/28370501/a-fluorescent-labeled-phosphono-bisbenzguanidine-as-an-activity-based-probe-for-matriptase
#9
Daniela Häußler, Anna-Christina Schulz-Fincke, Anna-Madeleine Beckmann, Aline Keils, Erik Gilberg, Martin Mangold, Jürgen Bajorath, Marit Stirnberg, Torsten Steinmetzer, Michael Gütschow
Activity-based probes are compounds that exclusively form covalent bonds with active enzymes. They can be utilized to profile enzyme activities in vivo, to identify target enzymes and to characterize their function. The design of a new activity-based probe for matriptase, a member of the type II transmembrane serine proteases, is based on linker-connected bis-benzguanidines. An amino acid, introduced as linker, bears the coumarin fluorophore. Moreover, an incorporated phosphonate allows for a covalent interaction with the active-site serine...
March 28, 2017: Chemistry: a European Journal
https://www.readbyqxmd.com/read/28368394/inhibition-of-cyclooxygenase-2-mediated-matriptase-activation-contributes-to-the-suppression-of-prostate-cancer-cell-motility-and-metastasis
#10
C-J Ko, S-W Lan, Y-C Lu, T-S Cheng, P-F Lai, C-H Tsai, T-W Hsu, H-Y Lin, H-Y Shyu, S-R Wu, H-H Lin, P-W Hsiao, C-H Chen, H-P Huang, M-S Lee
Chronic inflammation plays an important role in cancer development and progression. Cyclooxygenases-2 (COX-2) is a key enzyme in generating prostaglandins causing inflammation, is often found to be overexpressed in prostate cancer (PCa) and is correlated with PCa cell invasion and metastasis. We aim to investigate the molecular mechanism of how COX-2 promotes PCa cell invasion and metastasis and to evaluate the effect of COX-2 inhibitors in a selected model of PCa progression. Our results showed that the expression of COX-2 and Interleukin 1β (IL-1β) was upregulated in highly invasive PCa cells and was correlated with the activated levels of membrane-anchored serine protease matriptase...
April 3, 2017: Oncogene
https://www.readbyqxmd.com/read/28362108/alpha1-antitrypsin-deficient-macrophages-have-increased-matriptase-mediated-proteolytic-activity
#11
Karina Krotova, George W Marek, Rejean L Wang, George Aslanidi, Brad E Hoffman, Nazli Khodayari, Farshid N Rouhani, Mark L Brantly
Alpha1-antitrypsin (AAT) deficiency-associated emphysema is largely attributed to insufficient inhibition of neutrophil elastase released from neutrophils. Correcting AAT levels by augmentation therapy only slows disease progression; the absence of full recovery indicates a more complex process of lung destruction. Because alveolar macrophages (Mɸ) express AAT, we propose that the expression and intracellular accumulation of mutated Z-AAT (the most common mutation is called Z) compromise Mɸ function and contribute to emphysema development...
March 31, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28358853/a-peptide-based-approach-to-evaluate-the-adaptability-of-influenza-a-virus-to-humans-based-on-its-hemagglutinin-proteolytic-cleavage-site
#12
Marco R Straus, Gary R Whittaker
Cleavage activation of the hemagglutinin (HA) protein by host proteases is a crucial step in the infection process of influenza A viruses (IAV). However, IAV exists in eighteen different HA subtypes in nature and their cleavage sites vary considerably. There is uncertainty regarding which specific proteases activate a given HA in the human respiratory tract. Understanding the relationship between different HA subtypes and human-specific proteases will be valuable in assessing the pandemic potential of circulating viruses...
2017: PloS One
https://www.readbyqxmd.com/read/28294512/hfe-mutations-and-iron-in-hemodialysis-patients
#13
REVIEW
Luca Valenti, Serena Pelusi
In chronic hemodialysis patients, a disruption in iron metabolism ranging from absolute to functional deficiency, with compartmentalization of this metal into macrophages, is often observed. Chronic inflammation indeed often causes an upregulation of the iron hormone hepcidin, thereby reducing iron absorption and availability to the erythron. We systematically reviewed the literature on the role of genetic risk factors on iron metabolism in hemodialysis. In this setting, mutations in the HFE gene of hereditary hemochromatosis may confer an adaptive benefit by decreasing hepcidin release, thus improving iron availability to erythropoiesis, anemia control, and the response to erythropoiesis stimulating agents and iron itself, and reducing the side effects of these therapies...
June 2017: Hemodialysis International
https://www.readbyqxmd.com/read/28219045/modulating-the-selectivity-of-matriptase-2-inhibitors-with-unnatural-amino-acids
#14
Catherine St-Georges, Antoine Désilets, François Béliveau, Mariana Ghinet, Sébastien P Dion, Éloic Colombo, Pierre-Luc Boudreault, Rafael J Najmanovich, Richard Leduc, Éric Marsault
Matriptase-2, a type II transmembrane serine protease (TTSP), is expressed in the liver and regulates iron homeostasis via the cleavage of hemojuvelin. Matriptase-2 emerges as an attractive target for the treatment of conditions associated with iron overload, such as hemochromatosis or beta-thalassemia. Starting from the crystal structure of its closest homolog matriptase, we constructed a homology model of matriptase-2 in order to further optimize the selectivity of serine trap peptidomimetic inhibitors for matriptase-2 vs matriptase...
March 31, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28125689/selective-inhibition-of-prostasin-in-human-enterocytes-by-the-integral-membrane-kunitz-type-serine-protease-inhibitor-hai-2
#15
Frank Shiao, Li-Ching O Liu, Nanxi Huang, Ying-Jung J Lai, Robert J Barndt, Chun-Che Tseng, Jehng-Kang Wang, Bailing Jia, Michael D Johnson, Chen-Yong Lin
Mutations of hepatocyte growth factor activator inhibitor (HAI)-2 in humans cause sodium loss in the gastrointestinal (GI) tract in patients with syndromic congenital sodium diarrhea (SCSD). Aberrant regulation of HAI-2 target protease(s) was proposed as the cause of the disease. Here functional linkage of HAI-2 with two membrane-associated serine proteases, matriptase and prostasin was analyzed in Caco-2 cells and the human GI tract. Immunodepletion-immunoblot analysis showed that significant proportion of HAI-2 is in complex with activated prostasin but not matriptase...
2017: PloS One
https://www.readbyqxmd.com/read/28094766/matriptase-mediated-cleavage-of-epcam-destabilizes-claudins-and-dysregulates-intestinal-epithelial-homeostasis
#16
Chuan-Jin Wu, Xu Feng, Michael Lu, Sohshi Morimura, Mark C Udey
Congenital tufting enteropathy (CTE) is a severe autosomal recessive human diarrheal disorder with characteristic intestinal epithelial dysplasia. CTE can be caused by mutations in genes encoding EpCAM, a putative adhesion molecule, and HAI-2, a cell surface protease inhibitor. A similar phenotype occurs in mice whose intestinal epithelial cells (IECs) fail to express the tight junction-associated protein claudin-7. EpCAM stabilizes claudin-7 in IECs, and HAI-2 regulates the cell surface serine protease matriptase, a known modifier of intestinal epithelial physiology...
February 1, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28073521/current-status-of-iron-metabolism-clinical-and-therapeutic-implications
#17
REVIEW
Susana Conde Diez, Ricardo de Las Cuevas Allende, Eulogio Conde García
Hepcidin is the main regulator of iron metabolism and a pathogenic factor in iron disorders. Hepcidin deficiency causes iron overload, whereas hepcidin excess causes or contributes to the development of iron-restricted anaemia in chronic inflammatory diseases. We know the mechanisms involved in the synthesis of hepcidin and, under physiological conditions, there is a balance between activating signals and inhibitory signals that regulate its synthesis. The former include those related to plasmatic iron level and also those related to chronic inflammatory diseases...
March 3, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28045393/recombinant-hepatocyte-growth-factor-activator-inhibitor-1-expression-in-drosophila-s2-cells-purification-and-crystallization
#18
Min Liu, Cai Yuan, Yunbin Jiang, Longguang Jiang, Mingdong Huang
Hepatocyte growth factor activator inhibitor 1 (HAI-1) is a multi-domain membrane-associated protease inhibitor that potently inhibits a variety of serine proteases such as hepatocyte growth factor activator and matriptase. Different truncates of HAI-1 show varying potencies for inhibition of target proteases, suggesting that the domain organization of HAI-1 plays a critical role in its function. Here, the soluble full-length extracellular part of HAI-1 (sHAI-1) was expressed using the Drosophila S2 insect-cell expression system...
January 1, 2017: Acta Crystallographica. Section F, Structural Biology Communications
https://www.readbyqxmd.com/read/28035363/adar2-functions-as-a-tumor-suppressor-via-editing-igfbp7-in-esophageal-squamous-cell-carcinoma
#19
Yuan-Bin Chen, Xiao-Yu Liao, Jiang-Bo Zhang, Fang Wang, Hai-De Qin, Lanjun Zhang, Yin Yao Shugart, Yi-Xin Zeng, Wei-Hua Jia
Esophageal squamous cell carcinoma (ESCC), one of the most aggressive cancers, is characterized by heterogeneous genetic and epigenetic changes. Recently, A-to-I RNA editing, catalyzed by adenosine deaminases acting on RNA (ADARs), was found to be aberrantly regulated during tumorigenesis. We previously reported that ADAR2 was downregulated in ESCC but its role was unclear. Thus, we report here that overexpression of ADAR2 can induce apoptosis in ESCC cell lines and inhibit tumor growth in vitro and in vivo...
February 2017: International Journal of Oncology
https://www.readbyqxmd.com/read/28032468/three-dimensional-culture-of-single-embryonic-stem-derived-neural-stem-progenitor-cells-in-fibrin-hydrogels-neuronal-network-formation-and-matrix-remodelling
#20
Ana R Bento, Pedro Quelhas, Maria J Oliveira, Ana P Pêgo, Isabel F Amaral
In an attempt to improve the efficacy of neural stem/progenitor cell (NSPC) based therapies, fibrin hydrogels are being explored to provide a favourable microenvironment for cell survival and differentiation following transplantation. In the present work, the ability of fibrin to support the survival, proliferation, and neuronal differentiation of NSPCs derived from embryonic stem (ES) cells under monolayer culture was explored. Single mouse ES-NSPCs were cultured within fibrin (fibrinogen concentration: 6 mg/ml) under neuronal differentiation conditions up to 14 days...
December 29, 2016: Journal of Tissue Engineering and Regenerative Medicine
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