protein

C1 Esterase Inhibitor

MBS318174

1 mg

270 USD

Native Protein

C1 Esterase Inhibitor

C1 Inhibitor (Esterase Inhibitor)

Human C1 Esterase Inhibitor

C1 inhibitor; Plasma protease C1 inhibitor; plasma protease C1 inhibitor; serpin G1; OTTHUMP00000197003; OTTHUMP00000197004; OTTHUMP00000197007; OTTHUMP00000235286; OTTHUMP00000235287; C1-inhibiting factor; C1 esterase inhibitor; complement component 1 inhibitor; serine/cysteine proteinase inhibitor clade G member 1; serpin peptidase inhibitor, clade G (C1 inhibitor), member 1; C1 esterase inhibitor; C1-inhibiting factor; Serpin G1

SERPING1; SERPING1; C1IN; C1NH; HAE1; HAE2; C1INH; C1IN; C1NH

IC1_HUMAN

Human Plasma

C1 Inhibitor (Esterase Inhibitor)

>95% pure (SDS-PAGE)

Purified, Lyophilized. Reconstitute with 166.6ul distilled water.

1.03mg/ml (prior to lyophilization)

Store at -80 degree C.

Inactivation: Not applicable.

Buffer: Lyophilized from 20mM Potassium phosphate, pH 7.0 containing 250mM Potassium chloride. Preservative: No. Lyophilized: Yes. Warnings: All human source materials have tested negative for HIV1 and HIV2 and is non-reactive for anti-HCV and anti-HBc antibodies and HBsAg. No test guarantees a product to be non-infectious. Therefore, all material derived from human fluids or tissues

Purified Enzymes

1197499

CAA30314

P05155

55,154 Da

Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Formation Of Fibrin Clot (Clotting Cascade) Pathway (106057); Formation Of Platelet Plug Pathway (106029); Hemostasis Pathway (106028); Intrinsic Pathway (106059); Pertussis Pathway (218111); Pertussis Pathway (218099); Platelet Activation Pathway (106034)

This gene encodes a highly glycosylated plasma protein involved in the regulation of the complement cascade. Its protein inhibits activated C1r and C1s of the first complement component and thus regulates complement activation. Deficiency of this protein is associated with hereditary angioneurotic oedema (HANE). Alternative splicing results in multiple transcript variants encoding the same isoform. [provided by RefSeq]

Function: Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein. Ref.21. Subunit structure: Binds to E.coli stcE which allows localization of SERPING1 to cell membranes thus protecting the bacteria against complement-mediated lysis. Interacts with MASP1. Ref.22 Ref.23 Ref.25. Subcellular location: Secreted. Post-translational modification: Highly glycosylated (49%) with N- and O-glycosylation. Ref.2 Ref.24 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31Can be proteolytically cleaved by E.coli stcE. Ref.23 Ref.25. Polymorphism: Chymotrypsin uses Ala-465 as its reactive site in normal plasma protease C1 inhibitor, and His-466 as its reactive site in the variant His-466. Involvement in disease: Defects in SERPING1 are the cause of hereditary angioedema (HAE) [. MIM:106100]; also called hereditary angioneurotic edema (HANE). HAE is an autosomal dominant disorder characterized by episodic local subcutaneous edema and submucosal edema involving the upper respiratory and gastrointestinal tracts. HAE due to C1 esterase inhibitor deficiency is comprised of two clinically indistinguishable forms. In HAE type 1, representing 85% of patients, serum levels of C1 esterase inhibitor are less than 35% of normal. In HAE type 2, the levels are normal or elevated, but the protein is non-functional. Ref.14 Ref.20 Ref.33 Ref.34 Ref.35 Ref.36 Ref.37 Ref.38 Ref.39 Ref.40 Ref.41 Ref.42 Ref.43 Ref.44 Ref.45. Sequence similarities: Belongs to the serpin family. Sequence caution: The sequence AAA53096.1 differs from that shown. Reason: Erroneous gene model prediction.

1 mg

270 USD