catalog number :
MBS696162
products type :
Recombinant Protein
products full name :
Human C1 Inhibitor
products short name :
[C1 Inhibitor]
products name syn :
[Plasma protease C1 inhibitor, C1 inh Esterase Protein, C1-inhibiting factor, Serpin G1]
other names :
[Plasma protease C1 inhibitor; Plasma protease C1 inhibitor; plasma protease C1 inhibitor; serpin G1; 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]
products gene name :
[C1]
other gene names :
[SERPING1; SERPING1; C1IN; C1NH; HAE1; HAE2; C1INH; C1IN; C1NH; C1 Inh; C1Inh]
uniprot entry name :
IC1_HUMAN
reactivity :
Species Reactivity: Human
sequence :
VEPILEVSS LPTTNSTTN SATKITANT TDEPTTQPT TEPTTQPTI QPTQPTTQL PTDSPTQPT TGSFCPGPV TLCSDLESH STEAVLGDA LVDFSLKLY HAFSAMKKV ETNMAFSPF SIASLLTQV LLGAGENTK TNLESILSY PKDFTCVHQ ALKGFTTKG VTSVSQIFH SPDLAIRDT FVNASRTLY SSSPRVLSN NSDANLELI NTWVAKNTN NKISRLLDS LPSDTRLVL LNAIYLSAK WKTTFDPKK TRMEPFHFK NSVIKVPMM NSKKYPVAH FIDQTLKAK VGQLQLSHN LSLVILVPQ NLKHRLEDM EQALSPSVF KAIMEKLEM SKFQPTLLT LPRIKVTTS QDMLSIMEK LEFFDFSYD LNLCGLTED PDLQVSAMQ HQTVLELTE TGVEAAAAS AISVARTLL VFEVQQPFL FVLWDQQHK FPVFMGRVY DPRA
purity :
>=95% by SDS-PAGE and HPLC analyses.
form :
Sterile filterd though a 0.2 micron filter. Lyophilized from 10mM Sodium Phosphate, pH 7.5.
storage stability :
Store at -20°C
other info1 :
Authenticity: Verified by N-terminal and Mass Spectometry analyses (when applicable).
other info2 :
Endotoxin: Endotoxin level is 50 is <=2.6 nM). Reconstitution: Centrifuge the vial prior to opening . Reconstitute in water to a concentration of 0.1-1.0 mg/mL . Do not vortex. For extended storage, it is recommended to further dilute in a buffer containing a carrier protein (example 0.1% BSA) and store in working aliquots at -20°C to -80°C.
products categories :
Apoptosis; Immunology; Inflammation
products description :
C1 Inhibitor is a member of the serpin family of structurally related proteins, and is the primary regulator of the immune complement system. C1Inhibitor is a protease inhibitor that functions to inhibit the complement system in order to prevent over-activation or spontaneous activation. Inhibition is achieved by binding to and irreversibly inhibiting the C1r and C1s proteases of the C1 complex, which has the effect of shutting down all subsequent downstream events in the complement activation cascade. C1inhibitor can also inhibit various other proteases, including Kallikrein, Factor XIa, and Factor XIIa. Deficiencies in C1inhibitor are the primary cause of hereditary angioedema (HAE, hereditary angioneurotic edema), a disease characterized by edema in the respiratory and gastrointestinal tracts. In certain clinical situations, the direct administration of C1inhibitor can be used to treat HAE and certain other conditions. Recombinant Human C1 Inhibitor is a highly glycosylated glycoprotein containing 445 amino acid residues (49.4kDa), corresponding to amino acids 56 - 500 of the C1 inhibitor precursor, and is fully functional in its ability to inhibit the C1 complex. Glycosylated C1 Inhibitor migrates at an apparent molecular weight of approximately 80-90 kDa by SDS PAGE analysis under reducing conditions.
ncbi pathways :
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); Hemostasis Pathway (106028); Intrinsic Pathway (106059); Pertussis Pathway (218111); Pertussis Pathway (218099); Platelet Activation, Signaling And Aggregation Pathway (106034); Platelet Degranulation Pathway (106050)
ncbi summary :
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]
uniprot summary :
SERPING1: a protein protease inhibitor (C1-inhibitor) that forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play an important role in regulating complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Mutations of the SERPING1 gene is associated with adult macular degeneration can also cause hereditary angioedema. Binds to E.coli stcE which allows localization of SERPING1 to cell membranes thus protecting the bacteria against complement-mediated lysis. Belongs to the serpin family. Protein type: Secreted, signal peptide; Secreted. Chromosomal Location of Human Ortholog: 11q12.1. Cellular Component: extracellular space; extracellular region. Molecular Function: serine-type endopeptidase inhibitor activity; protein binding. Biological Process: negative regulation of complement activation, lectin pathway; platelet activation; fibrinolysis; platelet degranulation; blood circulation; innate immune response; blood coagulation; complement activation, classical pathway; blood coagulation, intrinsic pathway; aging. Disease: Complement Component 4, Partial Deficiency Of; Angioedema, Hereditary, Type I