Matriptase 2 Polyclonal Antibody, AbBy Fluor-350 Conjugated

Catalogue Number: BS-8296R-BF350-BSS

Manufacturer:Bioss Inc
Type:Polyclonal Primary Antibody - Conjugated
Alias:Matriptase-2; Matriptase2; Membrane type serine proteinase 2; MTSP 2; MTSP2; PVAE354; TMPRSS 6; TMPRSS6; TMPS6_HUMAN; TMSP 6; TMSP6; Transmembrane protease serine 6; Type II Membrane Serine Proteinase 6.
Shipping Condition:Blue Ice
Unit(s): 100 ul
Host name: Rabbit
Clone:
Isotype: IgG
Immunogen: KLH conjugated synthetic peptide derived from human Matriptase 2
Application: ICC, IF, IHC-P, WB, IHC-Fr

Description

Description: 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); 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.

Additional Text

Gene Name

TMPRSS6

Gene ID

164656

Purification

Protein A purified

Concentration

1 ug/ul

Antibody Clonality

Polyclonal

Modification

Unmodified

Storage Note

Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Excitation

346nm/442nm