重组Anti-Factor H抗体[EPR6226] (ab124769)
![Western blot - Anti-Factor H antibody [EPR6226] (ab124769)](https://www.abcam.cn/ps/products/124/ab124769/Images/ab124769-138631-anti-factor-h-antibody-epr6226-western-blot.jpg "Western blot - Anti-Factor H antibody [EPR6226] (ab124769)")
Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR6226] to Factor H
- Suitable for: WB
- Reacts with: Human
Related conjugates and formulations
概述
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产品名称
Anti-Factor H抗体[EPR6226]
参阅全部 Factor H 一抗 -
描述
兔单克隆抗体[EPR6226] to Factor H -
宿主
Rabbit -
经测试应用
适用于: WBmore details
不适用于: Flow Cyt,ICC/IF,IHC-P or IP -
种属反应性
与反应: Human -
免疫原
Synthetic peptide within Human Factor H aa 900-1000. The exact sequence is proprietary.
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阳性对照
- A549, Human fetal lung, Human fetal liver, Human kidney and Human plasma lysates
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常规说明
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.
性能
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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
pH: 7.20
Preservative: 0.05% Sodium azide
Constituents: 40% Glycerol (glycerin, glycerine), 9.85% Tris glycine, 50% Tissue culture supernatant -
Concentration information loading... -
纯度
Protein A purified -
克隆
单克隆 -
克隆编号
EPR6226 -
同种型
IgG -
研究领域
相关产品
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Alternative Versions
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Isotype control
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Recombinant Protein
应用
The Abpromise guarantee
Abpromise™承诺保证使用ab124769于以下的经测试应用
“应用说明”部分 下显示的仅为推荐的起始稀释度；实际最佳的稀释度/浓度应由使用者检定。
| 应用 | Ab评论 | 说明 |
|---|---|---|
| WB |
1/1000 - 1/10000. Detects a band of approximately 180 kDa (predicted molecular weight: 139 kDa).
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| 说明 |
|---|
|
WB
1/1000 - 1/10000. Detects a band of approximately 180 kDa (predicted molecular weight: 139 kDa). |
应用说明
Is unsuitable for Flow Cyt,ICC/IF,IHC-P or IP.
靶标
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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
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数据库链接
- Entrez Gene: 3075 Human
- Omim: 134370 Human
- SwissProt: P08603 Human
- Unigene: 363396 Human
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别名
- adrenomedullin binding protein antibody
- age related maculopathy susceptibility 1 antibody
- AHUS 1 antibody
see all
图片
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Western blot - Anti-Factor H antibody [EPR6226] (ab124769)All lanes : Anti-Factor H antibody [EPR6226] (ab124769) at 1/1000 dilution
Lane 1 : A549 cell lysate
Lane 2 : Human fetal lung lysate
Lane 3 : Human fetal liver lysate
Lane 4 : Human kidney lysate
Lane 5 : Human plasma lysate
Lysates/proteins at 10 µg per lane.
Secondary
All lanes : Goat anti-Rabbit HRP at 1/2000 dilution
Predicted band size: 139 kDa -
![Anti-Factor H antibody [EPR6226] (ab124769)](https://www.abcam.cn/ps/products/124/ab124769/Images/ab124769-2-benefits-of-recombinant-antibodies.png)
Anti-Factor H antibody [EPR6226] (ab124769)
数据表及文件
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SDS download
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Datasheet download
文献 (0)
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