AAQ19269.1
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
55,769 Da
NCBI Official Full Name
C1 inhibitor mutant, partial
NCBI Official Synonym Full Names
serpin peptidase inhibitor, clade G (C1 inhibitor), member 1
NCBI Protein Information
plasma protease C1 inhibitor
UniProt Protein Name
Plasma protease C1 inhibitor
UniProt Synonym Protein Names
C1 esterase inhibitor; C1-inhibiting factor; Serpin G1
UniProt Synonym Gene Names
UniProt Entry Name
IC1_HUMAN
NCBI Summary for C1INH
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 Comments for C1INH
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 region; extracellular space
Molecular Function: protein binding; serine-type endopeptidase inhibitor activity
Biological Process: aging; blood circulation; blood coagulation; blood coagulation, intrinsic pathway; complement activation, classical pathway; fibrinolysis; innate immune response; negative regulation of complement activation, lectin pathway; platelet activation; platelet degranulation
Disease: Angioedema, Hereditary, Type I; Complement Component 4, Partial Deficiency Of
Research Articles on C1INH
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Products associated with C1INH elisa kit
Pathways associated with C1INH elisa kit
Diseases associated with C1INH elisa kit
Organs/Tissues associated with C1INH elisa kit
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