半岛bd体育手机客户端 说明
一般描述
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)
Coagulation factor VIII is a serum protein. This protein is made up of a heterodimer of peptides, including A1-A2-B-A3-C1-C2 domains. The F8 gene is located on the human chromosome at Xq28.
免疫原
F8 (NP_063916.1, 1 a.a. ~ 216 a.a) full-length human protein.
Sequence
MRIQDPGKVFFGNVDSSGIKHNIFNPPIIARYIRLHPTHYSIRSTLRMELMGCDLNSCSMPLGMESKAISDAQITASSYFTNMFATWSPSKARLHLQGRSNAWRPQVNNPKEWLQVDFQKTMKVTGVTTQGVKSLLTSMYVKEFLISSSQDGHQWTLFFQNGKVKVFQGNQDSFTPVVNSLDPPLLTRYLRIHPQSWVHQIALRMEVLGCEAQDLY
生化/生理作用
Coagulation factor VIII is involved in nucleating the assembly of multiprotein proteolytic complexes. It binds to the activated platelets at the site of vascular injury and activates factor X. Coagulation factor VIII acts as a co-factor that participates in the intrinsic pathway of blood coagulation. Mutations in the F8 gene are associated with Hemophilia A, an inherited bleeding disorder.
外形
Solution in phosphate buffered saline, pH 7.4
免责声明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
基本信息
| NACRES | NA.41 |
半岛bd体育手机客户端 性质
| 生物来源 | rabbit |
| 质量水平 | 100 |
| 偶联物 | unconjugated |
| 抗体形式 | purified immunoglobulin |
| antibody product type | primary antibodies |
| 克隆 | polyclonal |
| 形式 | buffered aqueous solution |
| 分子量 | antigen 24.6 kDa |
| species reactivity | human |
| technique(s) | western blot: 1 μg/mL |
| NCBI登记号 | NM_019863.2 |
| UniProt登记号 | P00451 |
| 运输 | dry ice |
| 储存温度 | ?20℃ |
| Gene Information | human ... F8(2157) |
安全信息
| 储存分类代码 | 12 - Non Combustible Liquids |
| WGK | WGK 3 |
| 闪点(F) | Not applicable |
| 闪点(C) | Not applicable |
Sigma-Aldrich
m.cnreagent.com
