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CFH P08603

补体因子H (H因子1)

蛋白质信息 (UniProt)

补体因子H (H因子1)

P08603

功能描述

糖蛋白,通过调节补体激活在维持平衡的免疫反应中发挥重要作用。作为补体的可溶性抑制剂,其与糖结构等自身标记物的结合可防止细胞表面的补体激活和放大(PubMed:21285368, PubMed:21317894, PubMed:25402769)。加速补体替代途径(AP) C3转化酶C3bBb的衰变,从而防止补体放大环路的核心参与者C3b的局部形成(PubMed:19503104, PubMed:21317894, PubMed:26700768)。作为丝氨酸蛋白酶因子I的辅因子,CFH还调节已沉积C3b的蛋白水解降解(PubMed:18252712, PubMed:23332154, PubMed:28671664)。此外,通过与特定受体相互作用介导多种细胞反应。例如,与CR3/ITGAM受体相互作用,从而介导人类中性粒细胞对不同病原体的粘附。进而,这些病原体被吞噬并消灭(PubMed:20008295, PubMed:9558116)。 {ECO:0000269|PubMed:18252712, ECO:0000269|PubMed:19503104, ECO:0000269|PubMed:20008295, ECO:0000269|PubMed:21285368, ECO:0000269|PubMed:21317894, ECO:0000269|PubMed:23332154, ECO:0000269|PubMed:25402769, ECO:0000269|PubMed:26700768, ECO:0000269|PubMed:28671664, ECO:0000269|PubMed:9558116}.; FUNCTION: (微生物感染) 在蚊虫中肠中,结合于恶性疟原虫配子母细胞表面,保护寄生虫免受替代补体途径介导的清除。 {ECO:0000269|PubMed:23332154}.

组织特异性

Expressed in the retinal pigment epithelium (at protein level) (PubMed:25136834). CFH is one of the most abundant complement components in blood where the liver is the major source of CFH protein in vivo. in addition, CFH is secreted by additional cell types including monocytes, fibroblasts, or endothelial cells (PubMed:2139673, PubMed:25136834, PubMed:2968404, PubMed:6444659).

亚细胞定位

Secreted.; SUBCELLULAR LOCATION: Note=(Microbial infection) In the mosquito midgut, localizes to P.falciparum (NF54 strain) macrogamete and young zygote cell membranes.

关键词

3D-structure Age-related macular degeneration Alternative splicing Complement alternate pathway Direct protein sequencing Disease variant Disulfide bond Glycoprotein Hemolytic uremic syndrome Host-virus interaction