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B6-F8 KO
製品ID :
I001219
系統:
C57BL/6JCya
状況:
説明:
The F8 gene encodes coagulation factor VIII (FVIII), a large plasma glycoprotein crucial for the intrinsic pathway of blood coagulation. This gene generates two alternatively spliced transcripts, with the larger isoform, variant a, forming a non-covalent complex with von Willebrand factor (vWF) that circulates in plasma. Under normal physiological conditions, when a blood vessel is injured, platelets and clotting factors aggregate at the site of injury to form a blood clot and prevent further bleeding. In this process, FVIII functions as a cofactor, working synergistically with activated factor IX (FIXa) to activate factor X (FX), subsequently generating fibrin and stabilizing the blood clot. Deficiency of the F8 gene leads to hemophilia A (HA), an X-linked recessive bleeding disorder that primarily affects males. Patients often present with spontaneous or post-traumatic bleeding that may be difficult to control, with severe cases risking disability or life-threatening complications due to internal bleeding and joint hemorrhage. Clinically, exogenous FVIII supplementation is effective in managing hemophilia A symptoms. In recent years, advances in gene therapy and gene editing have offered new hope for HA treatment, aiming for a long-term or permanent cure by repairing or replacing the defective F8 gene.
The B6-F8 KO mouse is a hemophilia A (HA) research model developed through gene-editing techniques, where the murine F8 gene, homologous to the human F8 gene, is knocked out. Studies have shown that homozygous F8 knockout mice are viable and develop normally [1]. Since the murine F8 gene is located on the X chromosome, hemizygous male and homozygous female B6-F8 KO mice exhibit a consistent phenotype with significantly lower FVIII activity compared to wild-type mice, prolonged clotting time, and a classic hemophilia A phenotype [1].
The F8 gene encodes coagulation factor VIII (FVIII), a large plasma glycoprotein crucial for the intrinsic pathway of blood coagulation. This gene generates two alternatively spliced transcripts, with the larger isoform, variant a, forming a non-covalent complex with von Willebrand factor (vWF) that circulates in plasma. Under normal physiological conditions, when a blood vessel is injured, platelets and clotting factors aggregate at the site of injury to form a blood clot and prevent further bleeding. In this process, FVIII functions as a cofactor, working synergistically with activated factor IX (FIXa) to activate factor X (FX), subsequently generating fibrin and stabilizing the blood clot. Deficiency of the F8 gene leads to hemophilia A (HA), an X-linked recessive bleeding disorder that primarily affects males. Patients often present with spontaneous or post-traumatic bleeding that may be difficult to control, with severe cases risking disability or life-threatening complications due to internal bleeding and joint hemorrhage. Clinically, exogenous FVIII supplementation is effective in managing hemophilia A symptoms. In recent years, advances in gene therapy and gene editing have offered new hope for HA treatment, aiming for a long-term or permanent cure by repairing or replacing the defective F8 gene.
The B6-F8 KO mouse is a hemophilia A (HA) research model developed through gene-editing techniques, where the murine F8 gene, homologous to the human F8 gene, is knocked out. Studies have shown that homozygous F8 knockout mice are viable and develop normally [1]. Since the murine F8 gene is located on the X chromosome, hemizygous male and homozygous female B6-F8 KO mice exhibit a consistent phenotype with significantly lower FVIII activity compared to wild-type mice, prolonged clotting time, and a classic hemophilia A phenotype [1].
Efnb2-flox
製品ID :
S-CKO-02157
系統:
C57BL/6JCya
状況:
説明:
Efnb2 is located on chromosome 8 of mice. SgRNA and ssDNA were designed using Nuclease Technology; Efnb2 conditional knockout mice were obtained by high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
Efnb2 is located on chromosome 8 of mice. SgRNA and ssDNA were designed using Nuclease Technology; Efnb2 conditional knockout mice were obtained by high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
Gabra3-KO
製品ID :
S-KO-02157
系統:
C57BL/6JCya
状況:
説明:
Gabra3 is located on chromosome X of mice. Nuclease Technology was used to design sgRNA; Gabra3 knockout mice were obtained by applying high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
Gabra3 is located on chromosome X of mice. Nuclease Technology was used to design sgRNA; Gabra3 knockout mice were obtained by applying high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
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