Human Complement Factor H/CFH Standard (人补体因子H (CFH) 标准品)

CFH 分子靶点信息概述

• 分子名：CFH, complement factor H
• 基因家族：Sushi domain containing; Complement system regulators and receptors
• 别名：HUS; FHL1; ARMS1; ARMD4
• 曾用名：HF; HF1; HF2
• 全称：beta-1H; H factor 2 (complement); age-related maculopathy susceptibility 1; H factor 1 (complement)

CFH 分子靶点综述

补体因子H，又称H因子1和CFH，是一种含唾液酸的糖蛋白，在调节补体介导的免疫系统中起着不可或缺的作用，该免疫系统参与微生物防御、免疫复合物处理和细胞程序性死亡。CFH作为补体系统替代途径的调节因子，通过确保相关的免疫应答作用于非宿主细胞的外来病原体发挥作用。CFH保护自身细胞免受补体激活，但不保护细菌和病毒。CFH的错误调节可能对处理外来感染或降低宿主细胞补体活性的能力产生不利影响。H因子基因突变与多种严重疾病有关，包括罕见的肾脏疾病溶血尿毒综合征(HUS)和膜增生性肾小球肾炎(MPGN)，也被称为致密物沉积病(DDD)，II型膜增生性肾小球肾炎或致密物沉积病，以及更常见的年龄相关性黄斑变性(AMD)。

人 Human CFH 分子靶点信息

• 分子名：CFH, complement factor H
• 别称：
  • adrenomedullin binding protein
  • age-related maculopathy susceptibility 1
  • AHUS1
  • AMBP1
  • ARMD4
  • ARMS1
  • beta-1-H-globulin
  • beta-1H
  • CFHL3
  • complement factor H, isoform b
  • factor H
  • factor H-like 1
  • FH
  • FHL1
  • H factor 1 (complement)
  • H factor 2 (complement)
  • HF
  • HF1
  • HF2
  • HUS
  • MGC88246
• 基因序列：NCBI_Gene: 3075
• 蛋白序列：UniProtKB: P08603

人 Human CFH靶点分子功能（预测）

Enables heparan sulfate proteoglycan binding activity; heparin binding activity; and identical protein binding activity. Involved in complement activation. Acts upstream of or within regulation of complement activation and regulation of complement-dependent cytotoxicity. Located in blood microparticle and extracellular exosome. Implicated in several diseases, including atypical hemolytic-uremic syndrome; eye disease (multiple); hemolytic-uremic syndrome; lupus nephritis; and systemic lupus erythematosus. Biomarker of amphetamine abuse; atypical hemolytic-uremic syndrome; glaucoma; psoriasis; and squamous cell carcinoma.