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货号： bs-0434R 基本售价： 380.0 元 规格： 20ul
- 规格：20ul
- 价格：380.00元
- 规格：50ul
- 价格：780.00元
- 规格：100ul
- 价格：1380.00元
- 规格：200ul
- 价格：2200.00元
产品信息
- 产品编号
- bs-0434R
- 英文名称
- factor VIII
- 中文名称
- 凝血因子8/第八凝血因子/第八因子相关抗原抗体
- 别 名
- coagulation factor VIII; Ahf; Antihemophilic factor; Coagulation factor VIII; Coagulation factor VIII procoagulent component; Coagulation factor VIIIc; Dna segment on chromosome x unique 1253 expressed sequence; Dxs1253e; F8; F8 protein;F8c; FactorVIII; FVIII; Hema coagulation factor VIIIc procoagulent component; Hemophilia a; Hemophilia classic; OTTHUMP00000061446; Procoagulant component; FⅧ-Ag; Factor VIIIa heavy chain, 200 kDa isoform; Factor VIIIa heavy chain, 92 kDa isoform; AHF; DXS1253E; F8C; FVIII; HEMA; FA8_HUMAN.
- 规格价格
- 50ul/780元购买 100ul/1380元购买 200ul/2200元购买 大包装/询价
- 说 明 书
- 50ul 100ul 200ul
- 研究领域
- 肿瘤 免疫学 生长因子和激素
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Rabbit,
- 产品应用
- ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 267kDa
- 细胞定位
- 细胞外基质 分泌型蛋白
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from rat factor VIII:401-500/2258
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- PubMed
- PubMed
- 产品介绍
- background:
This gene encodes coagulation factor VIII, which participates in the intrinsic pathway of blood coagulation; factor VIII is a cofactor for factor IXa which, in the presence of Ca+2 and phospholipids, converts factor X to the activated form Xa. This gene produces two alternatively spliced transcripts. Transcript variant 1 encodes a large glycoprotein, isoform a, which circulates in plasma and associates with von Willebrand factor in a noncovalent complex. This protein undergoes multiple cleavage events. Transcript variant 2 encodes a putative small protein, isoform b, which consists primarily of the phospholipid binding domain of factor VIIIc. This binding domain is essential for coagulant activity. Defects in this gene results in hemophilia A, a common recessive X-linked coagulation disorder. [provided by RefSeq, Jul 2008].
Function:
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.
Subunit:
Interacts with vWF. vWF binding is essential for the stabilization of F8 in circulation.
Subcellular Location:
Secreted, extracellular space.
Post-translational modifications:
Sulfation on Tyr-1699 is essential for binding vWF.
DISEASE:
Hemophilia A (HEMA) [MIM:306700]: A disorder of blood coagulation characterized by a permanent tendency to hemorrhage. About 50% of patients have severe hemophilia resulting in frequent spontaneous bleeding into joints, muscles and internal organs. Less severe forms are characterized by bleeding after trauma or surgery. Note=The disease is caused by mutations affecting the gene represented in this entry. Of particular interest for the understanding of the function of F8 is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8 in their plasma (at least 30% of normal), but the protein is non-functional; i.e. the F8 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.
Similarity:
Belongs to the multicopper oxidase family.
Contains 3 F5/8 type A domains.
Contains 2 F5/8 type C domains.
Contains 6 plastocyanin-like domains.
SWISS:
Q7TN96
Gene ID:
2157
Database links:Entrez Gene: 403875 Dog
Entrez Gene: 2157 Human
Entrez Gene: 14069 Mouse
Entrez Gene: 397339 Pig
Omim: 300841 Human
SwissProt: O18806 Dog
SwissProt: P00451 Human
SwissProt: Q06194 Mouse
SwissProt: P12263 Pig
Unigene: 654450 Human
Unigene: 1805 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Antigen （又称：凝结因子VIII,抗血友病因子）第VIII因子抗体用于血管源性良性和恶性肿瘤的诊断，也用于遗传性血友病的研究。在正常动脉、静脉、毛细血管及心脏内细胞的血管内皮细胞上阳性表达。第Ⅷ因子相关抗原抗体在巨核细胞及血小板上也有表达。
第Ⅷ因子相关抗原:是一种糖蛋白，广泛存在于血管上皮、肝脏、脾窦上皮、及淋巴内皮细胞，是血管内皮细胞及其内源性良恶性肿瘤的特异性标记。主要用于血管原性良恶性肿瘤和血管肉瘤的诊断。少数副睾、子宫和输卵管的腺癌样瘤也有表达.
- 产品图片
Paraformaldehyde-fixed, paraffin embedded (rat brain tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (factor VIII) Polyclonal Antibody, Unconjugated (bs-0434R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-Factor VIII Polyclonal Antibody, Unconjugated(bs-0434R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining