Rabbit anti Coagulation Factor VIII (Fused Form) | MyBioSource MBS462035 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Rabbit anti Coagulation Factor VIII (Fused Form)

catalog :

MBS462035

quantity :

0.1 mg

price :

380 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human

application :

western blot, ELISA, immunohistochemistry

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS462035

products type :

Antibody

products full name :

Rabbit anti Coagulation Factor VIII (Fused Form)

products short name :

Coagulation Factor VIII (Fused form)

products name syn :

Rabbit anti-Coagulation Factor VIII (Fused Form); Factor VIII (FVIII)

other names :

coagulation factor VIII; Coagulation factor VIII; coagulation factor VIII; factor VIII F8B; OTTHUMP00000024269; OTTHUMP00000196174; antihemophilic factor; coagulation factor VIIIc; coagulation factor VIII, procoagulant component; Antihemophilic factor; AHF; Procoagulant component

other gene names :

F8; F8; AHF; F8B; F8C; HEMA; FVIII; DXS1253E; F8C

uniprot entry name :

FA8_HUMAN

host :

Rabbit

reactivity :

Human

specificity :

This antibody recognizes ~265 kDa of human coagulation Factor VIII protein. The other species are not tested

purity :

The Rabbit IgG is purified by Epitope Affinity Purification.

form :

This affinity purified antibody is supplied in sterile Phosphatebuffered saline (pH7.2) containing antibody stabilizer

concentration :

Size: 100 ug/200 ul

storage stability :

The antibodies are stable for 12 months from date of receipt when stored at -20 degree C to -70 degree C. The antibodies can be stored at 2 degree C-8 degree C for three month without detectable loss of activity. Avoid repeated freezing-thawing cycles.

tested application :

ELISA, Western Blot, Immunohistochemistry

app notes :

ELISA: 0.01-0.1 ug/ml

other info1 :

Antigen Preparation: A synthetic peptide corresponding to fused portion of human FVIII protein surrounding to HQREI domain with depletion of cleavage terminus.

products description :

The Coagulation Factor VIII (FVIII) is a glycoprotein essential for the intrinsic pathway of blood coagulation. A mature form of FVIII is a single-chain, 2351 amino acid polypeptide with a MW 265kDa. The FVIII can be activated by proteolytic cleavage intracellularly into a two-chain heterodimer, a heavy-chain and light-chain. The development of anti-factor VIII (FVIII) antibodies is currently one of the most serious complications in the treatment of haemophilia A patients due to the nature and properties of anti-FVIII antibodies, their mechanism of action, their neutralization by anti-idiotypic antibodies, and the role of T cells in FVIII inhibitor formation.

products references :

Brooks, MB. et al. Indirect carrier detection of canine haemophilia A using factor VIII microsatellite markers. Anim Genet, 2008 Jun; 39 (3): 278-83.

ncbi gi num :

182383

ncbi acc num :

AAA52420.1

ncbi gb acc num :

M88628.1

uniprot acc num :

P00451

ncbi mol weight :

265 kDa

ncbi pathways :

Blood Clotting Cascade Pathway (198840); Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Formation Of Fibrin Clot (Clotting Cascade) Pathway (106057); Formation Of Platelet Plug Pathway (106029); Hemostasis Pathway (106028); Intrinsic Pathway (106059); Platelet Activation Pathway (106034); Platelet Degranulation Pathway (106050)

ncbi summary :

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]

uniprot summary :

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 structure: Interacts with vWF. vWF binding is essential for the stabilization of F8 in circulation. Ref.10 Ref.11. Subcellular location: Secreted extracellular space. Domain: Domain F5/8 type C 2 is responsible for phospholipid-binding and essential for factor VIII activity. Post-translational modification: Sulfation on Tyr-1699 is essential for binding vWF. Involvement in disease: Defects in F8 are the cause of 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=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. Ref.18 Ref.19 Ref.20 Ref.21 Ref.22 Ref.23 Ref.24 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31 Ref.32 Ref.33 Ref.34 Ref.36 Ref.37 Ref.38 Ref.39 Ref.40 Ref.41 Ref.42 Ref.43 Ref.44 Ref.45 Ref.46 Ref.47 Ref.48 Ref.49 Ref.50 Ref.51 Ref.52 Ref.53 Ref.54 Ref.55 Ref.56 Ref.57 Ref.58 Ref.59 Ref.60 Ref.61 Ref.62 Ref.63 Ref.64 Ref.65 Ref.66 Ref.67 Ref.68 Ref.69 Ref.70 Ref.71 Ref.72 Ref.73 Ref.74 Ref.75 Ref.76 Ref.77 Ref.78 Ref.79 Ref.80 Ref.82 Ref.83 Ref.84 Ref.85 Ref.86 Ref.87 Ref.88 Ref.89 Ref.90 Ref.91 Ref.92 Ref.93 Ref.94 Ref.95 Ref.96 Ref.97. Pharmaceutical use: Available under the names Kogenate (Bayer) and Recombinate (Baxter and American Home Products). Used to treat hemophilia A. Sequence similarities: 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. Mass spectrometry: Molecular mass is 1367.6 Da from positions 356 - 378. Determined by ESI. Nonsulfated. Ref.8Molecular mass is 1407.4 Da from positions 356 - 378. Determined by ESI. Sulfated. Ref.8Molecular mass is 2975.4 Da from positions 400 - 424. Determined by ESI. Nonsulfated. Ref.8Molecular mass is 3024 Da from positions 727 - 752. Determined by ESI. Nonsulfated. Ref.8Molecular mass is 3104 Da from positions 727 - 752. Determined by ESI. Monosulfated. Ref.8Molecular mass is 3183.5 Da from positions 727 - 752. Determined by ESI. Disulfated. Ref.8Molecular mass is 3262.5 Da from positions 727 - 752. Determined by ESI. Trisulfated. Ref.8Molecular mass is 2460.9 Da from positions 1672 - 1692. Determined by ESI. Nonsulfated. Ref.8Molecular mass is 2540.7 Da from positions 1672 - 1692. Determined by ESI. Sulfated. Ref.8Molecular mass is 2000.7 Da from positions 1691 - 1708. Determined by ESI. Nonsulfated. Ref.8Molecular mass is 2080.7 Da from positions 1691 - 1708. Determined by ESI. Sulfated. Ref.8

size1 :

0.1 mg

price1 :

380 USD

more info or order :

MyBioSource product webpage