Molecular weight (DA)
55kD
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.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/40000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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
Protein name
Plasma protease C1 inhibitor
Other name
SERPING1; C1IN; C1NH; Plasma protease C1 inhibitor; C1 Inh; C1Inh; C1 esterase inhibitor; C1-inhibiting factor; Serpin G1
Fields
>>Complement and coagulation cascades;>>Pertussis
Human protein sequence Database
P05155
Mouse protein sequence database
P97290
Rat protein sequence database
Cellular localization
Secreted.
Tissue expression
Blood,Brain,Foreskin,Heart,Liver,Plasma,Uterus,
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