The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
WB: 1/1000 when using colorimetric substrates such as BCIP/NBT, and 1/5000 for chemiluminescent substrates. Predicted molecular weight: 89 kDa.
Note: Glycosylation and other posttranslational modifications make matriptase 2 run at higher than expected mass on reduced SDS PAGE gels.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
Serine protease which hydrolyzes a range of proteins including type I collagen, fibronectin and fibrinogen. Can also activate urokinase-type plasminogen activator with low efficiency. May play a specialized role in matrix remodeling processes in liver. Required to sense iron deficiency. Overexpression suppresses activation of the HAMP promoter.
Involvement in disease
Defects in TMPRSS6 are the cause of iron-refractory iron deficiency anemia (IRIDA) [MIM:206200]; also known as hypochromic microcytic anemia with defect in iron metabolism or hereditary iron-handling disorder or pseudo-iron-deficiency anemia. Key features include congenital hypochromic microcytic anemia, very low mean corpuscular erythrocyte volume, low transferrin saturation, abnormal iron absorption characterized by no hematologic improvement following treatment with oral iron, and abnormal iron utilization characterized by a sluggish, incomplete response to parenteral iron.
Belongs to the peptidase S1 family. Contains 2 CUB domains. Contains 3 LDL-receptor class A domains. Contains 1 peptidase S1 domain.
Cytoplasmic domain mediates HAMP suppression via proximal promoter element(s).
Gurieva I et al. Erythropoietin administration increases splenic erythroferrone protein content and liver TMPRSS6 protein content in rats. Blood Cells Mol Dis64:1-7 (2017).
Read more (PubMed: 28282554) »
Frýdlová J et al. Effect of Erythropoietin, Iron Deficiency and Iron Overload on Liver Matriptase-2 (TMPRSS6) Protein Content in Mice and Rats. PLoS One11:e0148540 (2016).
Read more (PubMed: 26845567) »