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B6-Fgfr3*neoY367C
製品ID :
C001745
系統:
C57BL/6NCya
状況:
説明:
The FGFR3 gene encodes Fibroblast Growth Factor Receptor 3, a transmembrane receptor tyrosine kinase that plays a crucial role in regulating cell growth, differentiation, and apoptosis. It is widely expressed in various tissues, including the brain, kidney, testis, lung, small intestine, and liver, but is particularly important in cells forming bones, especially within the growth plate of cartilage [1]. The Fdfr3*Y367C mutation, which corresponds to the human Y373C mutation (a gain-of-function mutation), leads to constitutive activation of the FGFR3 protein. This overactivity disrupts normal chondrocyte proliferation and differentiation, impairing endochondral ossification and linear bone growth [2]. As a result, this mutation is significantly associated with severe skeletal dysplasias, including Thanatophoric Dysplasia type I (TDI) and Achondroplasia (ACH), the most common form of short-limbed dwarfism, characterized by disproportionate short stature, macrocephaly, and other skeletal deformities [3]. Y373C is one of the common activating mutations of FGFR3, accounting for approximately 50% of patients with thanatophoric dysplasia (TD-type I), but a lower proportion in the more prevalent achondroplasia (ACH) (ACH is primarily dominated by the G380R mutation). In reported literature, the Fgfr3*Y367C mutation is typically used to construct mouse models in a heterozygous form, which corresponds to the heterozygous nature of this mutation in human clinical patients. Its dominant-negative effect is sufficient to cause the disease. Heterozygous mice have an average lifespan of 6-8 weeks and exhibit severe disease phenotypes [4].
B6-Fgfr3*neoY367C mice are obtained by introducing the Y367C mutation into the mouse Fgfr3 gene using gene editing technology. Internal preliminary data show that homozygous B6-Fgfr3*neoY367C mice die at 3 weeks. This model can be used to study the mechanisms and therapeutic approaches for diseases such as achondroplasia (ACH) and thanatophoric dysplasia (TD).
The FGFR3 gene encodes Fibroblast Growth Factor Receptor 3, a transmembrane receptor tyrosine kinase that plays a crucial role in regulating cell growth, differentiation, and apoptosis. It is widely expressed in various tissues, including the brain, kidney, testis, lung, small intestine, and liver, but is particularly important in cells forming bones, especially within the growth plate of cartilage [1]. The Fdfr3*Y367C mutation, which corresponds to the human Y373C mutation (a gain-of-function mutation), leads to constitutive activation of the FGFR3 protein. This overactivity disrupts normal chondrocyte proliferation and differentiation, impairing endochondral ossification and linear bone growth [2]. As a result, this mutation is significantly associated with severe skeletal dysplasias, including Thanatophoric Dysplasia type I (TDI) and Achondroplasia (ACH), the most common form of short-limbed dwarfism, characterized by disproportionate short stature, macrocephaly, and other skeletal deformities [3]. Y373C is one of the common activating mutations of FGFR3, accounting for approximately 50% of patients with thanatophoric dysplasia (TD-type I), but a lower proportion in the more prevalent achondroplasia (ACH) (ACH is primarily dominated by the G380R mutation). In reported literature, the Fgfr3*Y367C mutation is typically used to construct mouse models in a heterozygous form, which corresponds to the heterozygous nature of this mutation in human clinical patients. Its dominant-negative effect is sufficient to cause the disease. Heterozygous mice have an average lifespan of 6-8 weeks and exhibit severe disease phenotypes [4].
B6-Fgfr3*neoY367C mice are obtained by introducing the Y367C mutation into the mouse Fgfr3 gene using gene editing technology. Internal preliminary data show that homozygous B6-Fgfr3*neoY367C mice die at 3 weeks. This model can be used to study the mechanisms and therapeutic approaches for diseases such as achondroplasia (ACH) and thanatophoric dysplasia (TD).
Fgfr3-Y367C(neo-del)
製品ID :
C001952
系統:
C57BL/6NCya
状況:
説明:
The FGFR3 gene encodes Fibroblast Growth Factor Receptor 3, a transmembrane receptor tyrosine kinase that plays a crucial role in regulating cell growth, differentiation, and apoptosis. It is widely expressed in various tissues, including the brain, kidney, testis, lung, small intestine, and liver, but is particularly important in cells forming bones, especially within the growth plate of cartilage [1]. The Fgfr3*Y367C mutation, which corresponds to the human Y373C mutation (a gain-of-function mutation), leads to constitutive activation of the FGFR3 protein. This overactivity disrupts normal chondrocyte proliferation and differentiation, impairing endochondral ossification and linear bone growth [2]. As a result, this mutation is significantly associated with severe skeletal dysplasias, including Thanatophoric Dysplasia type I (TDI) and Achondroplasia (ACH), the most common form of short-limbed dwarfism, characterized by disproportionate short stature, macrocephaly, and other skeletal deformities [3]. Y373C is one of the common activating mutations of FGFR3, accounting for approximately 50% of patients with thanatophoric dysplasia (TD-type I), but a lower proportion in the more prevalent achondroplasia (ACH) (ACH is primarily dominated by the G380R mutation). In reported literature, the Fgfr3*Y367C mutation is typically used to construct mouse models in a heterozygous form, which corresponds to the heterozygous nature of this mutation in human clinical patients. Its dominant-negative effect is sufficient to cause the disease [4].
Fgfr3-Y367C(neo-del) mice are a neo‑free disease model generated by crossing Fgfr3‑neoY367C mice (catalog No.: C001745) with Flpo mice. Internal data indicate that these double-heterozygous offspring begin to die at postnatal day 11 (P11) and exhibit typical dwarfism phenotypes, characterized by reduced overall body size, shortened long bones, craniofacial skeletal abnormalities, and decreased trunk and rib dimensions. This model can be utilized to investigate the pathogenesis and therapeutic strategies for achondroplasia (ACH) and thanatophoric dysplasia (TD).
The FGFR3 gene encodes Fibroblast Growth Factor Receptor 3, a transmembrane receptor tyrosine kinase that plays a crucial role in regulating cell growth, differentiation, and apoptosis. It is widely expressed in various tissues, including the brain, kidney, testis, lung, small intestine, and liver, but is particularly important in cells forming bones, especially within the growth plate of cartilage [1]. The Fgfr3*Y367C mutation, which corresponds to the human Y373C mutation (a gain-of-function mutation), leads to constitutive activation of the FGFR3 protein. This overactivity disrupts normal chondrocyte proliferation and differentiation, impairing endochondral ossification and linear bone growth [2]. As a result, this mutation is significantly associated with severe skeletal dysplasias, including Thanatophoric Dysplasia type I (TDI) and Achondroplasia (ACH), the most common form of short-limbed dwarfism, characterized by disproportionate short stature, macrocephaly, and other skeletal deformities [3]. Y373C is one of the common activating mutations of FGFR3, accounting for approximately 50% of patients with thanatophoric dysplasia (TD-type I), but a lower proportion in the more prevalent achondroplasia (ACH) (ACH is primarily dominated by the G380R mutation). In reported literature, the Fgfr3*Y367C mutation is typically used to construct mouse models in a heterozygous form, which corresponds to the heterozygous nature of this mutation in human clinical patients. Its dominant-negative effect is sufficient to cause the disease [4].
Fgfr3-Y367C(neo-del) mice are a neo‑free disease model generated by crossing Fgfr3‑neoY367C mice (catalog No.: C001745) with Flpo mice. Internal data indicate that these double-heterozygous offspring begin to die at postnatal day 11 (P11) and exhibit typical dwarfism phenotypes, characterized by reduced overall body size, shortened long bones, craniofacial skeletal abnormalities, and decreased trunk and rib dimensions. This model can be utilized to investigate the pathogenesis and therapeutic strategies for achondroplasia (ACH) and thanatophoric dysplasia (TD).
Zbtb8a-KO
製品ID :
S-KO-14184
系統:
C57BL/6JCya
状況:
説明:
Zbtb8a is located on chromosome 4 of mice. Nuclease Technology will be used to design sgRNA; Zbtb8a knockout mice will be obtained by applying high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
Zbtb8a is located on chromosome 4 of mice. Nuclease Technology will be used to design sgRNA; Zbtb8a knockout mice will be obtained by applying high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
Card19-flox
製品ID :
S-CKO-14184
系統:
C57BL/6JCya
状況:
説明:
Card19 is located on chromosome 13 of mice. SgRNA and ssDNA will be designed using Nuclease Technology; Card19 conditional knockout mice will be obtained by high-throughput electroporation of fertilized eggs. After sexual maturity, sperm will be collected for cryopreservation.
Card19 is located on chromosome 13 of mice. SgRNA and ssDNA will be designed using Nuclease Technology; Card19 conditional knockout mice will be obtained by high-throughput electroporation of fertilized eggs. After sexual maturity, sperm will be collected for cryopreservation.
Fgfr3-KO
製品ID :
S-KO-02051
系統:
C57BL/6JCya
状況:
説明:
Fgfr3 is located on chromosome 5 of mice. Nuclease Technology was used to design sgRNA; Fgfr3 knockout mice were obtained by applying high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
Fgfr3 is located on chromosome 5 of mice. Nuclease Technology was used to design sgRNA; Fgfr3 knockout mice were obtained by applying high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
Fgfr3-flox
製品ID :
S-CKO-02416
系統:
C57BL/6JCya
状況:
説明:
Fgfr3 is located on chromosome 5 of mice. SgRNA and ssDNA were designed using Nuclease Technology; Fgfr3 conditional knockout mice were obtained by high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
Fgfr3 is located on chromosome 5 of mice. SgRNA and ssDNA were designed using Nuclease Technology; Fgfr3 conditional knockout mice were obtained by high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
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