Factor V Antibody Pair | F5 antibody pair
Factor V, human
NCBI and Uniprot Product Information
NCBI Description
This gene encodes an essential cofactor of the blood coagulation cascade. This factor circulates in plasma, and is converted to the active form by the release of the activation peptide by thrombin during coagulation. This generates a heavy chain and a light chain which are held together by calcium ions. The activated protein is a cofactor that participates with activated coagulation factor X to activate prothrombin to thrombin. Defects in this gene result in either an autosomal recessive hemorrhagic diathesis or an autosomal dominant form of thrombophilia, which is known as activated protein C resistance. [provided by RefSeq, Oct 2008]
Uniprot Description
Function: Central regulator of hemostasis. It serves as a critical cofactor for the prothrombinase activity of factor Xa that results in the activation of prothrombin to thrombin.
Enzyme regulation: Inhibited by SERPINA5. Ref.11 Ref.13 Ref.18 Ref.20
Subunit structure: Factor Va, the activated form of factor V, is composed of a heavy chain and a light chain, non-covalently bound. The interaction between the two chains is calcium-dependent. Forms heterodimer with SERPINA5.
Subcellular location: Secreted
By similarity.
Tissue specificity: Plasma.
Domain: Domain B contains 35 x 9 AA tandem repeats, and 2 x 17 AA repeats.
Post-translational modification: Thrombin activates factor V proteolytically to the active cofactor, factor Va (formation of a heavy chain at the N-terminus and a light chain at the C-terminus).Sulfation is required for efficient thrombin cleavage and activation and for full procoagulant activity.Activated protein C inactivates factor V and factor Va by proteolytic degradation.Phosphorylation sites are present in the extracellular medium.
Involvement in disease: Factor V deficiency (FA5D) [MIM:227400]: A blood coagulation disorder leading to an hemorrhagic diathesis known as parahemophilia.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.35 Ref.38Thrombophilia due to activated protein C resistance (THPH2) [MIM:188055]: A hemostatic disorder due to defective degradation of factor V by activated protein C. It is characterized by a poor anticoagulant response to activated protein C resulting in tendency to thrombosis.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.31 Ref.36 Ref.37 Ref.39 Ref.41Budd-Chiari syndrome (BDCHS) [MIM:600880]: A syndrome caused by obstruction of hepatic venous outflow involving either the hepatic veins or the terminal segment of the inferior vena cava. Obstructions are generally caused by thrombosis and lead to hepatic congestion and ischemic necrosis. Clinical manifestations observed in the majority of patients include hepatomegaly, right upper quadrant pain and abdominal ascites. Budd-Chiari syndrome is associated with a combination of disease states including primary myeloproliferative syndromes and thrombophilia due to factor V Leiden, protein C deficiency and antithrombin III deficiency. Budd-Chiari syndrome is a rare but typical complication in patients with polycythemia vera.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry.Ischemic stroke (ISCHSTR) [MIM:601367]: A stroke is an acute neurologic event leading to death of neural tissue of the brain and resulting in loss of motor, sensory and/or cognitive function. Ischemic strokes, resulting from vascular occlusion, is considered to be a highly complex disease consisting of a group of heterogeneous disorders with multiple genetic and environmental risk factors.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. Ref.40Pregnancy loss, recurrent, 1 (RPRGL1) [MIM:614389]: A common complication of pregnancy, resulting in spontaneous abortion before the fetus has reached viability. The term includes all miscarriages from the time of conception until 24 weeks of gestation. Recurrent pregnancy loss is defined as 3 or more consecutive spontaneous abortions.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. Ref.19
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.
Sequence caution: The sequence ABD23003.1 differs from that shown. Reason: Erroneous gene model prediction. The sequence CAI23065.1 differs from that shown. Reason: Erroneous gene model prediction.
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Product Notes
The F5 f5 (Catalog #AAA512183) is an Antibody Pair and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "Factor V, Antibody Pair" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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