NP_000053.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
plasma protease C1 inhibitor
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 SERPING1
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 SERPING1
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
Product References and Citations for anti-SERPING1 antibody
Park,K.H., Mol. Vis. 15, 200-207 (2009) Gosswein,T., Cytogenet. Genome Res. 121 (3-4), 181-188 (2008)
Research Articles on SERPING1
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Products associated with anti-SERPING1 antibody
Diseases associated with anti-SERPING1 antibody
Organs/Tissues associated with anti-SERPING1 antibody
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