該基因編碼肽酶s1蛋白家族的一個成員。編碼蛋白是補體系統c1復合體中的一個蛋白水解亞單位。補體系統在先天性免疫反應中起中介作用，最終引發吞噬、炎癥和細菌細胞壁破裂該基因突變與埃勒斯-丹洛綜合征有關。[由RefSeq提供，2018年12月]
This gene encodes a member of the peptidase S1 protein family. The encoded protein is a proteolytic subunit in the complement system C1 complex. The complement system acts as a mediator in the innate immune response by ultimately triggering phagocytosis, inflammation, and rupturing the bacterial cell wall. Mutations in this gene are associated with Ehlers-Danlos Syndrome. [provided by RefSeq, Dec 2018]