THE SERINE PROTEASE MATRIPTASE-2 (TMPRSS6) INHIBITS HEPCIDIN ACTIVATION BY CLEAVING MEMBRANE HEMOJUVELIN
Explore this paper's citation graph
Summary
It is confirmed that matriptase-2 lacking the serine protease domain, identified in the anemic Mask mouse (matriptase-2MASK), is fully inactive and that mutant R774C found in patients with genetic iron deficiency has decreased inhibitory activity.
- Type
- article
- Published
- 2008-10-30
- Cited by
- 577
- References
- 28
- Access
- Open access
- OpenAlex
- https://openalex.org/W2012956876
- Semantic Scholar
- https://api.semanticscholar.org/CorpusID:35765051
Keywords
Hepcidin, TMPRSS6, Serine protease, Cell biology, Proteases
References
- The type II transmembrane serine protease matriptase-2--identification, structural features, enzymology, expression pattern and potential roles.
- Defective targeting of hemojuvelin to plasma membrane is a common pathogenetic mechanism in juvenile hemochromatosis.
- Hepcidin Regulates Cellular Iron Efflux by Binding to Ferroportin and Inducing Its Internalization
- Matriptase-2, a Membrane-bound Mosaic Serine Proteinase Predominantly Expressed in Human Liver and Showing Degrading Activity against Extracellular Matrix Proteins*
- Zebrafish as a model for defining the functional impact of mammalian ferroportin mutations.
- Autosomal recessive ichthyosis with hypotrichosis caused by a mutation in ST14, encoding type II transmembrane serine protease matriptase.
- STAT3 is required for IL-6-gp130-dependent activation of hepcidin in vivo.
- Mutations in TMPRSS6 cause iron-refractory iron deficiency anemia (IRIDA)
- Understanding iron homeostasis through genetic analysis of hemochromatosis and related disorders.
- Interleukin-6 induces hepcidin expression through STAT3.
- Bone morphogenetic protein signaling by hemojuvelin regulates hepcidin expression
- Posttranslational processing of hepcidin in human hepatocytes is mediated by the prohormone convertase furin.
- Defective Intracellular Trafficking of Uromodulin Mutant Isoforms
- STAT3 mediates hepatic hepcidin expression and its inflammatory stimulation.
- A role for membrane-type serine protease (MT-SP1) in intestinal epithelial turnover.
- Evidence for a Matriptase-Prostasin Proteolytic Cascade Regulating Terminal Epidermal Differentiation*
- The epidermal barrier function is dependent on the serine protease CAP1/Prss8
- The Serine Protease TMPRSS6 Is Required to Sense Iron Deficiency
- Mutations in HFE2 cause iron overload in chromosome 1q–linked juvenile hemochromatosis
- Regulation of iron homeostasis by the hypoxia-inducible transcription factors (HIFs).
Cited by
- The iron cycle in chronic kidney disease (CKD): from genetics and experimental models to CKD patients.
- Functional diversity of TMPRSS6 isoforms and variants expressed in hepatocellular carcinoma cell lines
- Modulation of hepcidin as therapy for primary and secondary iron overload disorders: pre-clinical models and approaches
- Exploring the Mechanisms by which Increasing Dietary Protein Induces Iron Transporter Expression and Improves Intestinal Iron Absorption
- Iron excretion in iron dextran-overloaded mice.
- Régulation de l'hepcidine et le rôle de la lipocaline 2 dans l'homéostasie du fer / Novel insights into the regulation of hepcidin and the role of lipocalin 2 in iron homeostasis
- Roles Of Tea Consumption & Co-Fortification In Iron Nutrition
- Inflammation neither increases hepatic hepcidin nor affects intestinal (59)Fe-absorption in two murine models of bowel inflammation, hemizygous TNF(ΔARE/+) and homozygous IL-10(-/-) mice.
- A genome-wide meta-analysis identifies 22 loci associated with eight hematological parameters in the HaemGen consortium
- The Cutting Edge: Membrane Anchored Serine Protease Activities in the Pericellular Microenvironment
- DEVELOPMENT OF A MASS SPECTROMETRY DIAGNOSTIC ASSAY TO MONITOR HEPCIDIN LEVELS IN HEMOCHROMATOSIS PATIENTS
- Matriptase-2 and the biological behaviours of prostate cancer cells : a possible role for beta-catenin
- The role of adipocytes in the modulation of iron metabolism in obesity
- Repulsive Guidance Molecules (RGMs) and Neogenin in Bone Morphogenetic Protein (BMP) signaling
- The regulation of iron transport
- Is EPO therapy able to correct iron deficiency anaemia caused by matriptase‐2 deficiency?
- Iron-deficiency anemia.
- Relationship Between Iron and Erythropoiesis
- A Systems Biology Approach to Iron Metabolism
- Inhibitors of Matriptase‐2 Based on the Trypsin Inhibitor SFTI‐1
Related papers
- Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6.
- Erythroferrone Regulates Hepcidin Expression Independently of Matriptase 2
- Matriptase-2 regulates iron homeostasis primarily by setting the basal levels of hepatic hepcidin expression through a nonproteolytic mechanism
- Iron and hepcidin: a story of recycling and balance.
- Type II transmembrane serine proteases
- Covalent activity-based probes for imaging of serine proteases.
- Microbial serine protease inhibitors and their therapeutic applications.
- On serine protease