C1 Inhibitor, Polyclonal Antibody

C1 Inhibitor, human

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 11; NC_000011.9 (57365027..57382326). Location: 11q12-q13.1

Polyclonal

Goat

Prior to conjugation, this antibody was specific for C1 esterase inhibitor as demonstrated by immunoelectrophoresis and ELISA.

Peroxidase conjugated IgG

Store between -10 and -20 degree C. Product will become viscous but will not freeze. Avoid storage in frost-free freezers. Keep vial tightly capped. Allow product to warm to room temperature and gently mix before use. Avoid exposure to sodium azide as this is an inhibitor of peroxidase activity.

Small volumes of anti-SERPING1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This product is suitable as a source of peroxidase conjugated antibodies to C1 esterase inhibitor.

55,154 Da[Similar Products]

serpin peptidase inhibitor, clade G (C1 inhibitor), member 1

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

Plasma protease C1 inhibitor

C1IN; C1NH  [Similar Products]

IC1_HUMAN

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.

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