概述

  • 产品名称Anti-Factor H抗体
    参阅全部 Factor H 一抗
  • 描述
    山羊多克隆抗体to Factor H
  • 特异性This antibody is expected to recognize isoform a (NP_000177.2) only.
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide:

    HLVPDRKKDQYK

    , corresponding to Internal sequence amino acids 577-588 of Human Factor H

  • 阳性对照
    • A431 lysate. Human lung lysate.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液Preservative: 0.02% Sodium Azide
    Constituents: 0.5% BSA, Tris buffered saline, pH 7.3
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 纯化说明Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

相关产品

应用

Our Abpromise guarantee covers the use of ab36134 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
WB Use a concentration of 0.03 - 0.1 µg/ml. Detects a band of approximately 170 kDa (predicted molecular weight: 139 kDa).

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 图像

  • Anti-Factor H antibody (ab36134) at 0.03 µg/ml + A431 lysate (RIPA buffer, 30µg total protein per lane)

    Predicted band size : 139 kDa
    Observed band size : 170 kDa (why is the actual band size different from the predicted?)


    Primary incubated for 1 hour. Detected by western blot using chemiluminescence.
  • Anti-Factor H antibody (ab36134) at 1/2000 dilution + Human HepG2 whole cell lysate at 5 µg

    Secondary
    An HRP-conjugated donkey anti-goat IgG polyclonal at 1/2500 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 139 kDa
    Observed band size : 150 kDa (why is the actual band size different from the predicted?)

    This image is courtesy of an anonymous Abreview

    See Abreview

Anti-Factor H antibody (ab36134)参考文献

This product has been referenced in:
  • Lehr S  et al. Identification and validation of novel adipokines released from primary human adipocytes. Mol Cell Proteomics : (2011). WB ; Human . Read more (PubMed: 21947364) »

See 1 Publication for this product

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Thank you for confirming these details and for your cooperation.
I would like to remind you that our products are guaranteed to work in the applications and in the species listed on the product datasheet. As shown on www.abcam.com/ab36134, the ant...

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Thank you for taking time to complete our questionnaire and for contacting us. I am sorry to hear this antibody is not providing satisfactory results.
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Es tut mir leid zu hören, dass Sie Probleme mit diesem Antikörper haben.
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Abcam guarantees this product to work in the species/application used in this Abreview.
Application Western blot
Sample Human Cell lysate - whole cell (HepG2)
Loading amount 5 µg
Specification HepG2
Gel Running Conditions Reduced Denaturing (10% gel)
Blocking step BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 24°C
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Abcam user community

Verified customer

提交于 Mar 19 2010

Thank you for your enquiry. We have not tested ab36134 in human serum, only in A431 lysate so unfortunately we do not know what band pattern is given in human serum samples. With the A431 lysate there was only 1 band at around 150-160 kDa as can been...

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"