The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application notesWB: 1/1000 when using colorimetric substrates such as BCIP/NBT, and 1/5000 for chemiluminescent substrates. Predicted molecular weight: 89 kDa.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
FunctionSerine 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.
Tissue specificityLiver specific.
Involvement in diseaseDefects 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.
Sequence similaritiesBelongs to the peptidase S1 family. Contains 2 CUB domains. Contains 3 LDL-receptor class A domains. Contains 1 peptidase S1 domain.
DomainCytoplasmic domain mediates HAMP suppression via proximal promoter element(s).