Anti-F8 antibody produced in rabbit

产品说明

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

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