Factor VIII 抗体
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北京 101111
Quick Overview for Factor VIII 抗体 (ABIN111959)
抗原
See all Factor VIII (F8) 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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特异性
- This antibody recognizes the 83 kDa light chain of Factor VIII. It does not cross-react with Von Willebrand factor.
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纯化方法
- Affinity Chromatography on Protein A
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免疫原
- Purified Human Factor VIII
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亚型
- IgG2a
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应用备注
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ELISA. Functional assays.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user. -
限制
- 仅限研究用
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浓度
- 1.0 mg/mL
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缓冲液
- PBS, pH 7.2 containing 0.09 % Sodium Azide as preservative
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储存液
- Sodium azide
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注意事项
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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注意事项
- Avoid repeated freezing and thawing.
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储存条件
- 4 °C/-20 °C
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储存方法
- Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
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- Factor VIII (F8) (Coagulation Factor VIII (F8))
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背景
- Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa. It is an extracellular factor. Defects in F8 are the cause of hemophilia A (HEMA). HEMA is a common recessive X linked coagulation disorder. The frequency of hemophilia A is 1-2 in 10,000 male births in all ethnic groups. About 50 % of patients have severe hemophilia A with F8C activity less than 1 % of normal, they have frequent spontaneous bleeding into joints, muscles and internal organs. Moderately severe hemophilia A occurs in about 10 % of patients, F8C activity is 2-5 % of normal, and there is bleeding after minor trauma. Mild hemophilia A, which occurs in 30-40 % of patients, is associated with F8C activity of 5-30 % and bleeding occurs only after significant trauma or surgery. Of particular interest for the understanding of the function of F8C is the category of CRM (cross-reacting material) positive patients (approximately 5 %) that have considerable amount of F8C in their plasma (at least 30 % of normal), but the protein is nonfunctional, i.e., the F8C activity is much less than the plasma protein level. CRM reduced is another category of patients in which the F8C antigen and activity are reduced to approximately the same level. Most mutations are CRM negative, and probably affect the folding and stability of the protein.Synonyms: AHF, Antihemophilic factor, F8C, Procoagulant component
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基因ID
- 2157
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NCBI登录号
- NP_000123
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UniProt
- P00451
抗原
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