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
Other Designations:
complement component 1 inhibitor,plasma protease C1 inhibitor,serine/cysteine proteinase inhibitor clade G member 1
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