Lonoctocog alfa is a recombinant Factor VIII used to treat hemophilia A to control bleeding.
>99% by SDS-Page and HPLC analysis
<0.001 EU per 1 μg of the peptide by the LAL method
For the treatment of hemophilia A, von Willebrand disease and Factor XIII deficiency.
Examples of Clinical Use:
Hemophilia A, von Willebrand disease and Factor XIII deficiency
Antihemophilic Factor binds factor IXa along with calcium and phospholipid, This complex converts factor X to factor Xa to facilitate clotting cascade.
Mechanism of action:
Antihemophilic factor (AHF) is a protein found in normal plasma which is necessary for clot formation. The administration of AHF provides an increase in plasma levels of AHF and can temporarily correct the coagulation defect of patients with hemophilia A (classical hemophilia).
Factor VIII protein (FVIII) is one of the important factors in the human coagulation system, consisting of a long heavy chain and a light chain. FVIII is mainly synthesized by liver cells, but can also be found in other tissues, such as vascular endothelial cells, lymphocytes, and fibroblasts. FVIII protein plays a very important role in blood coagulation, and the formation of blood clots requires activation of FVIII protein to activate other prothrombin, ultimately leading to the formation of fibrin.
The Function of Factor VIII Protein
FVIII protein has two main functions in the process of blood coagulation: firstly, it forms and maintains blood clots after vascular damage, and secondly, it resists bleeding diseases in the body.
In the case of vascular damage, FVIII protein is released and binds to another coagulation factor called FIX, forming a complex called prothrombin complex.
The prothrombin complex can activate another coagulation factor called FX, and a specific fragment of FX, FXa, can cleave fibrinogen and fibrinogen and convert them into fibrin, ultimately forming a blood clot. The defect or absence of FVIII can interfere with the formation of blood clots and lead to many hemorrhagic diseases.
If the quality or quantity of FVIII protein is deficient in the body, it can lead to severe bleeding in the human body. For example: in some cases of acquired defects, the quantity or quality of FVIII protein is significantly reduced, leading to serious bleeding problems in the early stage. This situation will be called hereditary Haemophilia, and it is an entry-level disease of blood coagulation caused by genetic defects.
Factor VIII protein related signaling pathway
The FVIII signaling pathway is a complex signaling system that involves many important components, such as endothelial cytokines (vWF), FVIII, FIX, platelets, fibrin, and other components that play important roles in this process. Short circuits in the FVIII signaling pathway or defects in any of these factors can lead to the failure or excessive formation of blood clots, leading to serious consequences such as blood stasis.
Factor VIII protein related diseases
There are mainly two kinds of FVIII protein and related diseases: hereditary Haemophilia and anti FVIII antibody disease.
The cause of hereditary Haemophilia can be the deletion, mutation or insufficient expression of FVIII gene, which is a serious blood coagulation disorder caused by factors such as gene silencing and activation, and is common in men. During the blood coagulation process, missing or defective FVIII protein can cause the formation of blood clots to fail, leading to mild to severe bleeding problems.
Anti FVIII antibody disease is another problem caused by the immune response in the body. Although FVIII protein can activate blood clotting complement, if some immune cells in the human immune system attack these FVIII proteins or their binding signaling substances that have no protective effect as foreign invading antibodies, it will lead to strong immune response and difficulty in restoring ability, leading to long-term bleeding conditions. In order to prevent this disease, many antiviral vaccines targeting the immune system have also been introduced in the market.
Potential applications of Factor VIII protein in medicine
Factor VIII protein has broad application prospects. First, it can be used in medicine to treat hereditary Haemophilia. These patients can use blood products made from FVIII protein to supplement the missing FVIII protein in their bodies. In addition, FVIII protein is also used to treat other bleeding related diseases, such as Rheumatoid arthritis, severe collagen disease or systemic lupus erythematosus.
FVIII protein has potential applications not only in medicine, but also in the food industry and biochemical fields. In the food industry, FVIII protein is used to prepare dairy products such as cheese, milk protein, and yogurt. In the field of biochemistry, FVIII protein can be used to study many biochemical processes, such as protein structure determination, new drug development, and interaction with biomolecules.
List of Factors VIII protein related drugs under research
Lonoctocog alfa: a recombinant Factor VIII used to treat hemophilia A to control bleeding.
BAY 1093884: a narrow angle vascular endothelial growth factor receptor (VEGF-R) and selective Factor VIIIa inhibitor, which can be used to treat Haemophilia and Deep vein thrombosis.
Emicizumab: an antibody with an artificial structure similar to Factor VIIIa, which is used to treat Haemophilia A and can replace the function of defective Factor VIIIa.
Fitusiran: a RNA interference agent that can inhibit the production of anticoagulant factors and is used to treat Haemophilia A or B.
AMT-060: A gene therapy drug, which is used to treat Haemophilia A by introducing healthy Factor VIII gene into patients.
CSL312: An antibody against Factor XIIa and Factor XIa, which can be used to prevent and treat thrombotic and hemorrhagic diseases.
Bispecific T cell engager (BiTE) technology: an artificial antibody that interacts with T cells and can stimulate the killing effect of T cells against Haemophilia.
Hemlibra: An artificial antibody developed by Roche that can bind to Factor IXa and Factor X to replace the missing Factor VIIIa and achieve coagulation.
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without prior written approval from Creative BioMart.
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