C1 Inhibitor Active Protein | SERPING1 active protein

Human C1 Inhibitor

Cat. Num. AAA692227

• Gene Names: SERPING1; C1IN; C1NH; HAE1; HAE2; C1INH
• Reactivity: Human
• Purity: >95% by SDS-PAGE & HPLC analysis
• Synonyms: C1 Inhibitor; Human C1 Inhibitor; Plasma protease C1; C1inh; C1 Esterase Protein; C1-inhibiting factor; Serpin G1; SERPING1 active protein

• Host: CHO cells
• Reactivity: Human
• Purity/Purification: >95% by SDS-PAGE & HPLC analysis
• Form/Format: Lyophilized
• Sequence: Protein Sequence: VEPILEVSSL PTTNSTTNSA TKITANTTDE PTTQPTTEPT TQPTIQ PTQP TTQLPTDSPT QPTTGSFCPG PVTLCSDLES HSTEAVLGDA L VDFSLKLYH AFSAMKKVET NMAFSPFSIA SLLTQVLLGA GENTKTN LES ILSYPKDFTC VHQALKGFTT KGVTSVSQIF HSPDLAIRDT FV NASRTLYS SSPRVLSNNS DANLELINTW VAKNTNNKIS RLLDSLPS DT RLVLLNAIYL SAKWKTTFDP KKTRMEPFHF KNSVIKVPMM NSK KYPVAHF IDQTLKAKVG QLQLSHNLSL VILVPQNLKH RLEDMEQAL S PSVFKAIMEK LEMSKFQPTL LTLPRIKVTT SQDMLSIMEK LEFF DFSYDL NLCGLTEDPD LQVSAMQHQT VLELTETGVE AAAASAISVA RTLLVFEVQQ PFLFVLWDQQ HKFPVFMGRV YDPRA
• Sequence Length: 500

• Species: Human
• Biological Activity: Measured by its ability to inhibit recombinant human complement component C1a cleavage of a colorimetric peptide substrate, N Carbobenzyloxy-Lys-ThioBenzyl ester (Z-K-SBzl). The expected IC50 is <= 2.6 nM.

Related Product Information for SERPING1 active protein

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.

Product Categories/Family for SERPING1 active protein

Recombinant protein; Cytokines & Growth Factors; C1 Inhibitor

NCBI and Uniprot Product Information

• NCBI GI #: 73858568
• NCBI GeneID: 710
• NCBI Accession #: NP_000053.2
• NCBI GenBank Nucleotide #: NM_000062.2
• UniProt Accession #: P05155; Q16304; Q547W3; Q59EI5; Q7Z455; Q96FE0; Q9UC49; A6NMU0; A8KAI9; B2R6L5; B4E1F0; B4E1H2
• Molecular Weight: 55,769 Da
• NCBI Official Full Name: plasma protease C1 inhibitor
• NCBI Official Synonym Full Names: serpin peptidase inhibitor, clade G (C1 inhibitor), member 1
• NCBI Official Symbol: SERPING1
• NCBI Official Synonym Symbols: C1IN; C1NH; HAE1; HAE2; C1INH
• NCBI Protein Information: plasma protease C1 inhibitor; serpin G1; C1-inhibiting factor; C1 esterase inhibitor; complement component 1 inhibitor; serine/cysteine proteinase inhibitor clade G member 1
• UniProt Protein Name: Plasma protease C1 inhibitor
• Protein Family: Plasma protease C1 inhibitor
• UniProt Gene Name: SERPING1
• UniProt Synonym Gene Names: C1IN; C1NH; C1 Inh; C1Inh
• UniProt Entry Name: IC1_HUMAN

NCBI Description

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 Description

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; Secreted, signal peptide

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; blood coagulation, intrinsic pathway; complement activation, classical pathway; aging

Disease: Angioedema, Hereditary, Type I; Complement Component 4, Partial Deficiency Of