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8 件の結果が “6010” で取得されました
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huRHO
製品ID :
C001396
系統:
C57BL/6JCya
状況:
説明:
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-4]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [5-10].
This strain is a mouse Rho gene humanized model, in which the mouse Rho gene is replaced by the human RHO gene. The protein encoded by the human gene is normally expressed in the mouse. Therefore, the structure and function of the retina of this model are identical to those of wild-type mice, and there is no visual defect. This model can be used to study visual signaling and retinitis pigmentosa (RP). Based on the self-developed technological innovation of TurboKnockout fusion BAC recombination, Cyagen can also provide popular point mutation disease models constructed based on this model. The data shows that B6J-hRHO-P23H mice carrying a human RHO pathogenic mutation constructed based on B6J-hRHO mice exhibit a distinct retinal abnormal phenotype. Additionally, Cyagen can provide customized services for different point mutations to meet the needs of a wide range of R&D personnel regarding the pharmacodynamics of retinitis pigmentosa (RP) and other preclinical needs.
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-4]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [5-10].
This strain is a mouse Rho gene humanized model, in which the mouse Rho gene is replaced by the human RHO gene. The protein encoded by the human gene is normally expressed in the mouse. Therefore, the structure and function of the retina of this model are identical to those of wild-type mice, and there is no visual defect. This model can be used to study visual signaling and retinitis pigmentosa (RP). Based on the self-developed technological innovation of TurboKnockout fusion BAC recombination, Cyagen can also provide popular point mutation disease models constructed based on this model. The data shows that B6J-hRHO-P23H mice carrying a human RHO pathogenic mutation constructed based on B6J-hRHO mice exhibit a distinct retinal abnormal phenotype. Additionally, Cyagen can provide customized services for different point mutations to meet the needs of a wide range of R&D personnel regarding the pharmacodynamics of retinitis pigmentosa (RP) and other preclinical needs.
huRHO(2)
製品ID :
C001646
系統:
C57BL/6JCya
状況:
説明:
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-4]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [5-10].
This strain is a mouse Rho gene humanized model, in which the mouse Rho gene is replaced by the human RHO gene. The protein encoded by the human gene is normally expressed in the mouse. Therefore, the structure and function of the retina of this model are identical to those of wild-type mice, and there is no visual defect. This model can be used to study visual signaling and retinitis pigmentosa (RP). Based on the self-developed technological innovation of TurboKnockout fusion BAC recombination, Cyagen can also provide popular point mutation disease models constructed based on this model. The data shows that huRHO-P23H mice carrying a human RHO pathogenic mutation constructed based on huRHO mice exhibit a distinct retinal abnormal phenotype. Additionally, Cyagen can provide customized services for different point mutations to meet the needs of a wide range of R&D personnel regarding the pharmacodynamics of retinitis pigmentosa (RP) and other preclinical needs.
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-4]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [5-10].
This strain is a mouse Rho gene humanized model, in which the mouse Rho gene is replaced by the human RHO gene. The protein encoded by the human gene is normally expressed in the mouse. Therefore, the structure and function of the retina of this model are identical to those of wild-type mice, and there is no visual defect. This model can be used to study visual signaling and retinitis pigmentosa (RP). Based on the self-developed technological innovation of TurboKnockout fusion BAC recombination, Cyagen can also provide popular point mutation disease models constructed based on this model. The data shows that huRHO-P23H mice carrying a human RHO pathogenic mutation constructed based on huRHO mice exhibit a distinct retinal abnormal phenotype. Additionally, Cyagen can provide customized services for different point mutations to meet the needs of a wide range of R&D personnel regarding the pharmacodynamics of retinitis pigmentosa (RP) and other preclinical needs.
huRHO-P23H
製品ID :
C001495
系統:
C57BL/6JCya
状況:
説明:
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-6]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [7-12].
This strain is a mouse Rho gene humanized model, in which the endogenous mouse Rho gene is replaced by the human RHO gene carrying a P23H mutation to express human retinal proteins in mice. Therefore, the abnormal protein encoded by the human gene was expressed in mice, resulting in abnormal retinal appearance and function and visual defects in this model. Based on the self-developed technological innovation of TurboKnockout fusion BAC recombination, Cyagen can also provide customized services for different point mutations to meet the needs of a wide range of R&D personnel regarding the pharmacodynamics of retinitis pigmentosa (RP) and other preclinical needs.
Mutations in the RHO gene can lead to rhodopsin-mediated autosomal dominant retinitis pigmentosa (RHO-adRP). In 25% of autosomal dominant inherited RP (adRP) cases, there are over 150 different RHO gene mutations. Notably, the P23H mutation is one of the most prevalent, accounting for 10% of adRP cases [2]. Previous studies have shown that mice carrying the heterozygous human RHO P23H mutation exhibit retinopathy and progressive retinal degeneration similar to the patient's disease process, which could be used for visual signaling and retinitis pigmentosa (RP) studies [3]. huRHO-P23H homozygous mice develop the disease earlier and have a more severe phenotype than heterozygous mice. Considering the uncertainty of growth and survival of homozygous mice due to late blindness, it is recommended to use huRHO-P23H heterozygous mice for experiments. However, homozygous mice may also be selected for research according to specific experimental needs.
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-6]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [7-12].
This strain is a mouse Rho gene humanized model, in which the endogenous mouse Rho gene is replaced by the human RHO gene carrying a P23H mutation to express human retinal proteins in mice. Therefore, the abnormal protein encoded by the human gene was expressed in mice, resulting in abnormal retinal appearance and function and visual defects in this model. Based on the self-developed technological innovation of TurboKnockout fusion BAC recombination, Cyagen can also provide customized services for different point mutations to meet the needs of a wide range of R&D personnel regarding the pharmacodynamics of retinitis pigmentosa (RP) and other preclinical needs.
Mutations in the RHO gene can lead to rhodopsin-mediated autosomal dominant retinitis pigmentosa (RHO-adRP). In 25% of autosomal dominant inherited RP (adRP) cases, there are over 150 different RHO gene mutations. Notably, the P23H mutation is one of the most prevalent, accounting for 10% of adRP cases [2]. Previous studies have shown that mice carrying the heterozygous human RHO P23H mutation exhibit retinopathy and progressive retinal degeneration similar to the patient's disease process, which could be used for visual signaling and retinitis pigmentosa (RP) studies [3]. huRHO-P23H homozygous mice develop the disease earlier and have a more severe phenotype than heterozygous mice. Considering the uncertainty of growth and survival of homozygous mice due to late blindness, it is recommended to use huRHO-P23H heterozygous mice for experiments. However, homozygous mice may also be selected for research according to specific experimental needs.
huRHO(2)-P23H
製品ID :
C001727
系統:
C57BL/6JCya
状況:
説明:
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-6]. Mutations in the RHO gene can lead to rhodopsin-mediated autosomal dominant retinitis pigmentosa (RHO-adRP). In 25% of autosomal dominant inherited RP (adRP) cases, there are over 150 different RHO gene mutations. Notably, the P23H mutation is one of the most prevalent, accounting for 10% of adRP cases [2]. Previous studies have shown that mice carrying the heterozygous human RHO P23H mutation exhibit retinopathy and progressive retinal degeneration similar to the patient's disease process, which could be used for visual signaling and retinitis pigmentosa (RP) studies [3]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [7-11].
This strain is a mouse Rho gene humanized model, in which the endogenous mouse Rho gene and Rho gene promoter are replaced by the human RHO gene carrying a P23H mutation and RHO gene promoter to express human retinal proteins in mice. Therefore, the abnormal protein encoded by the human gene was expressed in mice, resulting in abnormal retinal appearance and function and visual defects in this model. Based on the self-developed technological innovation of TurboKnockout fusion BAC recombination, Cyagen can also provide customized services for different point mutations to meet the needs of a wide range of R&D personnel regarding the pharmacodynamics of retinitis pigmentosa (RP) and other preclinical needs.
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-6]. Mutations in the RHO gene can lead to rhodopsin-mediated autosomal dominant retinitis pigmentosa (RHO-adRP). In 25% of autosomal dominant inherited RP (adRP) cases, there are over 150 different RHO gene mutations. Notably, the P23H mutation is one of the most prevalent, accounting for 10% of adRP cases [2]. Previous studies have shown that mice carrying the heterozygous human RHO P23H mutation exhibit retinopathy and progressive retinal degeneration similar to the patient's disease process, which could be used for visual signaling and retinitis pigmentosa (RP) studies [3]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [7-11].
This strain is a mouse Rho gene humanized model, in which the endogenous mouse Rho gene and Rho gene promoter are replaced by the human RHO gene carrying a P23H mutation and RHO gene promoter to express human retinal proteins in mice. Therefore, the abnormal protein encoded by the human gene was expressed in mice, resulting in abnormal retinal appearance and function and visual defects in this model. Based on the self-developed technological innovation of TurboKnockout fusion BAC recombination, Cyagen can also provide customized services for different point mutations to meet the needs of a wide range of R&D personnel regarding the pharmacodynamics of retinitis pigmentosa (RP) and other preclinical needs.
huRHO-P23H/huRHO
製品ID :
C001517
系統:
C57BL/6JCya
状況:
説明:
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-6]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [7-12].
This strain is a humanized model of the Rho gene with a heterozygous P23H mutation. It is obtained by mating homozygous B6J-hRHO mice (Catalog Number: C001396) with homozygous B6-hRHO-P23H mice (Catalog Number: C001495). In this model, the mouse Rho gene is replaced by the human RHO gene carrying the pathogenic mutation (P23H) and the human RHO gene without the mutation, respectively. The abnormal protein encoded by the mutant human gene is expressed in the mice. Therefore, the model exhibits abnormalities in the appearance and function of the retina, as well as visual defects. In addition, based on the technological innovation of TurboKnockout combined with BAC recombination developed independently, Cyagen Biosciences can also provide customized services for different point mutations based on B6-hRHO humanized mice to meet the experimental needs related to retinitis pigmentosa (RP) diseases. Mutations in the RHO gene are a major cause of RHO-mediated autosomal dominant retinitis pigmentosa (RHO-adRP). In 25% of autosomal dominant RP (adRP) cases, over 150 different RHO gene mutants have been identified. The P23H mutation is one of the most common causes of autosomal dominant retinitis pigmentosa, accounting for approximately 10% of adRP cases [2]. Previous studies have demonstrated that heterozygous mice carrying this mutation exhibit retinal pathology and progressive retinal degeneration similar to the disease progression in patients [3], making them valuable for studying visual signal transduction and retinitis pigmentosa (RP). Homozygous mice develop the disease earlier and have more severe phenotypes compared to heterozygous mice. Considering the uncertainty of the growth status and survival period of homozygous mice due to blindness in the later stage, it is generally recommended to use heterozygous mice (huRHO-P23H/huRHO, Catalog Number: C001517) for experiments.
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-6]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [7-12].
This strain is a humanized model of the Rho gene with a heterozygous P23H mutation. It is obtained by mating homozygous B6J-hRHO mice (Catalog Number: C001396) with homozygous B6-hRHO-P23H mice (Catalog Number: C001495). In this model, the mouse Rho gene is replaced by the human RHO gene carrying the pathogenic mutation (P23H) and the human RHO gene without the mutation, respectively. The abnormal protein encoded by the mutant human gene is expressed in the mice. Therefore, the model exhibits abnormalities in the appearance and function of the retina, as well as visual defects. In addition, based on the technological innovation of TurboKnockout combined with BAC recombination developed independently, Cyagen Biosciences can also provide customized services for different point mutations based on B6-hRHO humanized mice to meet the experimental needs related to retinitis pigmentosa (RP) diseases. Mutations in the RHO gene are a major cause of RHO-mediated autosomal dominant retinitis pigmentosa (RHO-adRP). In 25% of autosomal dominant RP (adRP) cases, over 150 different RHO gene mutants have been identified. The P23H mutation is one of the most common causes of autosomal dominant retinitis pigmentosa, accounting for approximately 10% of adRP cases [2]. Previous studies have demonstrated that heterozygous mice carrying this mutation exhibit retinal pathology and progressive retinal degeneration similar to the disease progression in patients [3], making them valuable for studying visual signal transduction and retinitis pigmentosa (RP). Homozygous mice develop the disease earlier and have more severe phenotypes compared to heterozygous mice. Considering the uncertainty of the growth status and survival period of homozygous mice due to blindness in the later stage, it is generally recommended to use heterozygous mice (huRHO-P23H/huRHO, Catalog Number: C001517) for experiments.
huRHO(2)-P23H/huRHO(2)
製品ID :
C001839
系統:
C57BL/6JCya
状況:
説明:
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-6]. Mutations in the RHO gene can lead to rhodopsin-mediated autosomal dominant retinitis pigmentosa (RHO-adRP). In 25% of autosomal dominant inherited RP (adRP) cases, there are over 150 different RHO gene mutations. Notably, the P23H mutation is one of the most prevalent, accounting for 10% of adRP cases [2]. Previous studies have shown that mice carrying the heterozygous human RHO P23H mutation exhibit retinopathy and progressive retinal degeneration similar to the patient's disease process, which could be used for visual signaling and retinitis pigmentosa (RP) studies [3]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [7-11].
huRHO(2)-P23H/huRHO(2) mouse is an F1 humanized model generated by crossing homozygous huRHO(2) mice (Catalog No.: C001646) with homozygous huRHO(2)-P23H mice (Catalog No.: C001727). This model carries two human RHO gene alleles, both with humanized promoter regions. One allele is the wild-type human RHO (without mutation), while the other is the human RHO carrying a pathogenic point mutation (P23H) (hRHO*P23H). The abnormal proteins encoded by the human genes are expressed in mice, resulting in abnormal retinal morphology, functional impairments, and visual defects in this model. Additionally, leveraging the technological innovation of independently developed TurboKnockout-fused BAC recombination, Cyagen Biosciences can provide customized services for different point mutations based on B6-hRHO humanized mice to meet the experimental needs of researchers, such as pharmacodynamic studies related to retinitis pigmentosa (RP).
Retinitis pigmentosa (RP) is a hereditary retinal disease with a global prevalence of approximately 1:5000-1:3000. RP is highly clinically and genetically heterogeneous, with mutations in the rhodopsin (RHO) gene causing approximately 25% of dominant RP [1]. The rhodopsin encoded by the RHO gene is closely associated with visual light transduction and GPCR downstream signals. Rhodopsin is essential for the transmission of light signals in the process of vision formation. Most RHO mutations lead to high levels of rhodopsin expression in photoreceptor cells, causing many mutant proteins to be abnormally located and aggregated in cells. This results in the apoptosis of photoreceptor cells, which cannot perform normal light signal transduction functions. Additionally, mutations in the RHO gene are associated with congenital stationary night blindness (CSNB) [2-6]. Mutations in the RHO gene can lead to rhodopsin-mediated autosomal dominant retinitis pigmentosa (RHO-adRP). In 25% of autosomal dominant inherited RP (adRP) cases, there are over 150 different RHO gene mutations. Notably, the P23H mutation is one of the most prevalent, accounting for 10% of adRP cases [2]. Previous studies have shown that mice carrying the heterozygous human RHO P23H mutation exhibit retinopathy and progressive retinal degeneration similar to the patient's disease process, which could be used for visual signaling and retinitis pigmentosa (RP) studies [3]. Current gene therapy targeting the RHO gene to treat retinitis pigmentosa includes ASO, CRISPR, and others. Applying fully humanized animal models will promote the further development of RHO-related potential therapies in clinical trials [7-11].
huRHO(2)-P23H/huRHO(2) mouse is an F1 humanized model generated by crossing homozygous huRHO(2) mice (Catalog No.: C001646) with homozygous huRHO(2)-P23H mice (Catalog No.: C001727). This model carries two human RHO gene alleles, both with humanized promoter regions. One allele is the wild-type human RHO (without mutation), while the other is the human RHO carrying a pathogenic point mutation (P23H) (hRHO*P23H). The abnormal proteins encoded by the human genes are expressed in mice, resulting in abnormal retinal morphology, functional impairments, and visual defects in this model. Additionally, leveraging the technological innovation of independently developed TurboKnockout-fused BAC recombination, Cyagen Biosciences can provide customized services for different point mutations based on B6-hRHO humanized mice to meet the experimental needs of researchers, such as pharmacodynamic studies related to retinitis pigmentosa (RP).
Cnst-KO
製品ID :
S-KO-06010
系統:
C57BL/6JCya
状況:
説明:
Cnst is located on chromosome 1 of mice. Nuclease Technology will be used to design sgRNA; Cnst knockout mice will be obtained by applying high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
Cnst is located on chromosome 1 of mice. Nuclease Technology will be used to design sgRNA; Cnst knockout mice will be obtained by applying high-throughput electroporation of fertilized eggs. After sexual maturity, sperm were collected for cryopreservation.
Wdpcp-flox
製品ID :
S-CKO-06010
系統:
C57BL/6JCya
状況:
説明:
Wdpcp is located on chromosome 11 of mice. SgRNA and ssDNA will be designed using Nuclease Technology; Wdpcp conditional knockout mice will be obtained by high-throughput electroporation of fertilized eggs. After sexual maturity, sperm will be collected for cryopreservation.
Wdpcp is located on chromosome 11 of mice. SgRNA and ssDNA will be designed using Nuclease Technology; Wdpcp conditional knockout mice will be obtained by high-throughput electroporation of fertilized eggs. After sexual maturity, sperm will be collected for cryopreservation.
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