Target：C1INH

Fields：Complement and coagulation cascades;Pertussis

Gene Name：SERPING1

Protein Name：Plasma protease C1 inhibitor

Human Gene Id：710

Human Swiss Prot No：P05155

Mouse Swiss Prot No：P97290

Immunogen：The antiserum was produced against synthesized peptide derived from human SERPING1. AA range:342-391

Specificity：C1INH Polyclonal Antibody detects endogenous levels of C1INH protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：SERPING1;C1IN;C1NH;Plasma protease C1 inhibitor;C1 Inh;C1Inh;C1 esterase inhibitor;C1-inhibiting factor;Serpin G1

Observed Band(KD)：55kD

Background： 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, Jul 2008],

Function：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.,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 coagul

Subcellular Location：Secreted.

Expression： Blood,Brain,Foreskin,Heart,Liver,Plasma,Uterus,