人 Complement factor B 抗体对
01AB02306
Complement factor B (CFB) is a crucial protein in humans, primarily involved in the alternative pathway of the complement system, which plays a vital role in immune responses. This 93-kDa single-chain polypeptide circulates in the blood and, upon activation, is cleaved by complement factor D into two fragments: the catalytic subunit Bb and the non-catalytic chain Ba. Bb acts as a serine protease that forms the C3 convertase when associated with C3b, facilitating the activation of the complement cascade and enhancing immune defense against pathogens. Mutations or deficiencies in CFB are linked to various diseases, including atypical hemolytic-uremic syndrome (aHUS) and age-related macular degeneration (AMD), where impaired complement regulation leads to increased susceptibility to infections and inflammatory conditions.
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技术参数
- 靶点基因名
- CFB
- 基因别名
- BF; BFD
- 蛋白别名
- complement factor B; Complement factor B
- UniProt
- P00751
- 物种
- Human
产品详情
- 靶点名称
- Complement factor B
- 蛋白名称
- complement factor B
- 蛋白别名
- Complement factor B; C3/C5 convertase; Glycine-rich beta glycoprotein; GBG; PBF2; Properdin factor B) [Cleaved into: Complement factor B Ba fragment; Complement factor B Bb fragment]
- 靶点基因名
- CFB
- 基因别名
- BF; BFD
- UniProt
- P00751
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Complement factor B (CFB) is a crucial protein in humans, primarily involved in the alternative pathway of the complement system, which plays a vital role in immune responses. This 93-kDa single-chain polypeptide circulates in the blood and, upon activation, is cleaved by complement factor D into two fragments: the catalytic subunit Bb and the non-catalytic chain Ba. Bb acts as a serine protease that forms the C3 convertase when associated with C3b, facilitating the activation of the complement cascade and enhancing immune defense against pathogens. Mutations or deficiencies in CFB are linked to various diseases, including atypical hemolytic-uremic syndrome (aHUS) and age-related macular degeneration (AMD), where impaired complement regulation leads to increased susceptibility to infections and inflammatory conditions.