Cloned (Comment) | Organism |
---|---|
gene TMPRSS6, expression of C-terminally c-Myc-tagged wild-type and truncated mutant enzymes in Hep-G2 cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
additional information | costruction of enzyme mutants with deletion of first 9 amino acids or the first 46 amino acids | Homo sapiens |
General Stability | Organism |
---|---|
low intracellular iron increases the stability of matriptase-2, overview | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
cell surface | the cytoplasmic domain of the enzyme contains an endocytosis motif that mediates the internalization of cell surface enzyme in a dynamin-dependent manner, Iron depletion does not affect the internalization of cell surface enzyme | Homo sapiens | 9986 | - |
membrane | a type II transmembrane serine protease | Homo sapiens | 16020 | - |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
additional information | low intracellular iron increases the stability of matriptase-2, overview | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
hemojuvelin + H2O | Homo sapiens | a glycosylphosphatidylinositol-linked membrane protein. inactivation | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | Q8IU80 | gene TMPRSS6 | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
hepatocyte | predominantly | Homo sapiens | - |
liver | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
hemojuvelin + H2O | inactivation | Homo sapiens | ? | - |
? | |
hemojuvelin + H2O | a glycosylphosphatidylinositol-linked membrane protein. inactivation | Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
More | the type II transmembrane protease is composed of a short cytoplasmic domain, a transmembrane domain, and a large extracellular domain, which contains a membrane-proximal stem region, a predicted activation domain, and a C-terminal catalytic domain | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
matriptase-2 | - |
Homo sapiens |
MT2 | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
7.4 | - |
assay at | Homo sapiens |
Organism | Comment | Expression |
---|---|---|
Homo sapiens | in Hep-G2 cells stably expressing the coding sequence of gene TMPRSS6, encoding the enzyme, incubation with apo-transferrin or the membrane-impermeable iron chelator deferoxamine mesylate salt, increases enzyme levels. This increase does not result from the inhibition of enzyme shedding from the cells | additional information |
Homo sapiens | the enzyme is up-regulated under iron deprivation | up |
General Information | Comment | Organism |
---|---|---|
malfunction | lack of the putative endocytosis motif in the cytoplasmic domain largely abolishes the sensitivity of the enzyme to iron depletion | Homo sapiens |
additional information | in Hep-G2 cells stably expressing the coding sequence of gene TMPRSS6, encoding the enzyme, incubation with apo-transferrin or the membrane-impermeable iron chelator deferoxamine mesylate salt, increases enzyme levels. This increase does not result from the inhibition of enzyme shedding from the cells | Homo sapiens |
physiological function | the enzyme suppresses the expression of hepatic hepcidin, an iron regulatory hormone, by cleaving membrane hemojuvelin into an inactive form. Hemojuvelin is a bone morphogenetic protein coreceptor. Regulation of the enzyme occurs at the level of protein degradation rather than by changes in the rate of internalization and translational or transcriptional mechanisms, the cytoplasmic enzyme domain is necessary for its regulation | Homo sapiens |