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Anti-C1INH Antibody

品牌：Antibodies

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規(guī)格：100μl

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Anti-C1INH Antibody

商品詳情參考文獻(xiàn) 相關(guān)資料

Overview

Name:	Anti-C1INH Antibody See all C1INH primary antibodies
Description:	Rabbit polyclonal antibody to C1INH
Applications:	WB, IHC
Reactivity:	Human
Immunogen:	Recombinant protein fragment contain a sequence corresponding to a region within amino acids 77 and 390 of Human SERPING1
Host:	Rabbit
Clonality:	Polyclonal
Conjugate:	Unconjugated
Purification:	Purified by antigen-affinity chromatography.
Formulation:	Supplied in 0.1M Tris-buffered saline with 10% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative.
Storage:	Store at -20?C for long term preservation (recommended). Store at 4?C for short term use.

Target

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.
Involvement in Disease:	Hereditary angioedema: An autosomal dominant disorder characterized by episodic local swelling involving subcutaneous or submucous tissue of the upper respiratory and gastrointestinal tracts, face, extremities, and genitalia. Hereditary angioedema due to C1 esterase inhibitor deficiency is comprised of two clinically indistinguishable forms. In hereditary angioedema type 1, serum levels of C1 esterase inhibitor are decreased, while in type 2, the levels are normal or elevated, but the protein is non-functional.
Sequence Similarities:	Belongs to the serpin family.
Post-Translational Modification:	Highly glycosylated (49%) with N- and O-glycosylation. O-glycosylated with core 1 or possibly core 8 glycans. N-glycan heterogeneity at Asn-25: Hex5HexNAc4 (minor), dHex1Hex5HexNAc4 (minor), Hex6HexNAc5 (major) and dHex1Hex6HexNAc5 (minor).
Cellular Location:	Secreted.
Database Links:	Entrez Gene: 710?Human Omim: 606860?Human SwissProt: P05155?Human Unigene: 384598?Human
Synonyms:	C1 esterase inhibitor Antibody C1 Inactivator Antibody C1 Inh Antibody C1 inhibiting factor Antibody C1 inhibitor Antibody C1-inhibiting factor Antibody C1IN Antibody C1Inh Antibody C1NH Antibody complement component 1 inhibitor Antibody esterase inhibitor Antibody HAE1 Antibody HAE2 Antibody IC1_HUMAN Antibody Plasma protease C1 inhibitor Antibody Serine (or cysteine) proteinase inhibitor clade G member 1 Antibody serine/cysteine proteinase inhibitor clade G member 1 Antibody Serpin G1 Antibody serpin peptidase inhibitor, clade G (C1 inhibitor), member 1 Antibody SERPING1 Antibody
Information:	Target information shown above is from the UniProt Consortium.