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Rabbit Anti-Factor VIII B chain  antibody (bs-10397R)
~~~促销,代码KX240301~~~
~~~促销,代码KX240302~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-10397R
英文名称 Factor VIII B chain
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 associated protein b; Coagulation factor VIII isoform b; Coagulation factor VIII procoagulent component; Coagulation factor VIIIc; Dna segment on chromosome x unique 1253 expressed sequence; Dxs1253e; F8; F8 protein; F8b; F8c; Factor VIII F8b; 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; Factor VIII B chain; AHF; DXS1253E; F8B; F8C; FVIII; HEMA; FA8_HUMAN.  
研究领域 肿瘤  心血管  免疫学  神经生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human
产品应用 WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 138kDa
细胞定位 细胞外基质 分泌型蛋白 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Factor VIII B chain: 1101-1200/2351 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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:
P00451

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



产品图片
Sample:Siha Cell (Human) Lysate at 40 ug
Primary: Anti-Factor VIII B chain(bs-10397R)at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 138kD
Observed band size: 135kD
Sample:
Lane 1: Hela (Human) Cell Lysate at 30 ug
Lane 2: U87MG (Human) Cell Lysate at 30 ug
Primary: Anti-Factor VIII B chain (bs-10397R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 110 kD
Observed band size: 110 kD
Paraformaldehyde-fixed, paraffin embedded (Human kidney); 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 B chain) Polyclonal Antibody, Unconjugated (bs-10397R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (human colon carcinoma); 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 B chain) Polyclonal Antibody, Unconjugated (bs-10397R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (human brain); 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 B chain) Polyclonal Antibody, Unconjugated (bs-10397R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (Human kidney); 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 B chain) Polyclonal Antibody, Unconjugated (bs-10397R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (human brain); 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 B chain) Polyclonal Antibody, Unconjugated (bs-10397R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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