Anti-Factor H抗体[63G5] (ab51043)

概述

  • 产品名称Anti-Factor H抗体[63G5]
    参阅全部 Factor H 一抗
  • 描述
    小鼠单克隆抗体[63G5] to Factor H
  • 经测试应用适用于: ELISA, IPmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length native protein purified from Human plasma

  • 阳性对照
    • Human plasma

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
  • 存储溶液Preservative: None
    Constituents: 50% Glycerol, PBS
  • Concentration information loading...
  • 克隆单克隆
  • 克隆编号63G5
  • 同种型IgG2b
  • 轻链类型kappa
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab51043 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
ELISA Use at an assay dependent dilution.
IP Use at an assay dependent dilution. Use 1µl/400µl lysate.

靶标

  • 功能Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway.
  • 组织特异性Expressed by the liver and secreted in plasma.
  • 疾病相关Genetic variations in CFH are associated with basal laminar drusen (BLD) [MIM:126700]; also known as drusen of Bruch membrane or cuticular drusen or grouped early adult-onset drusen. Drusen are extracellular deposits that accumulate below the retinal pigment epithelium on Bruch membrane. Basal laminar drusen refers to an early adult-onset drusen phenotype that shows a pattern of uniform small, slightly raised yellow subretinal nodules randomly scattered in the macula. In later stages, these drusen often become more numerous, with clustered groups of drusen scattered throughout the retina. In time these small basal laminar drusen may expand and ultimately lead to a serous pigment epithelial detachment of the macula that may result in vision loss.
    Defects in CFH are the cause of complement factor H deficiency (CFH deficiency) [MIM:609814]. CFH deficiency determines uncontrolled activation of the alternative complement pathway with consumption of C3 and often other terminal complement components. It is associated with a number of renal diseases with variable clinical presentation and progression, including membranoproliferative glomerulonephritis and atypical hemolytic uremic syndrome. CFH deficiency patients may show increased susceptibility to meningococcal infections.
    Defects in CFH are a cause of susceptibility to hemolytic uremic syndrome atypical type 1 (AHUS1) [MIM:235400]. An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Note=Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype.
    Genetic variation in CFH is associated with age-related macular degeneration type 4 (ARMD4) [MIM:610698]. ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane.
  • 序列相似性Contains 20 Sushi (CCP/SCR) domains.
  • 细胞定位Secreted.
  • Information by UniProt
  • 数据库链接
  • 别名
    • adrenomedullin binding protein antibody
    • age related maculopathy susceptibility 1 antibody
    • AHUS 1 antibody
    • AHUS1 antibody
    • AMBP 1 antibody
    • AMBP1 antibody
    • ARMD 4 antibody
    • ARMD4 antibody
    • ARMS 1 antibody
    • ARMS1 antibody
    • beta 1 H globulin antibody
    • beta 1H antibody
    • beta1H antibody
    • CFAH_HUMAN antibody
    • CFH antibody
    • CFHL 3 antibody
    • CFHL3 antibody
    • Complement factor H antibody
    • complement factor H, isoform b antibody
    • Factor H antibody
    • factor H like 1 antibody
    • FH antibody
    • FHL 1 antibody
    • FHL1 antibody
    • H factor 1 (complement) antibody
    • H factor 1 antibody
    • H factor 2 (complement) antibody
    • HF 1 antibody
    • HF 2 antibody
    • HF antibody
    • HF1 antibody
    • HF2 antibody
    • HUS antibody
    • MGC88246 antibody
    see all

Anti-Factor H antibody [63G5] 图像

  • Immunoprecipitation analysis of human plasma using anti-Factor H antibody ab51043, at 1µg for 400µl lysate.
    Lane 1: human plasma; Lane 2: Immunoprecipitate.

Anti-Factor H antibody [63G5] (ab51043)参考文献

ab51043 has not yet been referenced specifically in any publications.

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