Gene expression profile of the IL2RG gene depletion in pig spleen and thymus
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PROVIDER: PRJNA310855 | ENA |
REPOSITORIES: ENA
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Project description:Gene expression profile of the RAG2 gene depletion on pig spleen, thymus and lymph node
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Project description:Severe combined immunodeficiency (SCID) occurs in various species, including humans, at a frequency as high as one per 50,000 live births. Generally, SCID can be classified according to the cause of the immunodeficiency, and it includes impaired cytokine-mediated signalling, defective V(D)J recombination, impaired pre-T-cell receptor signalling, and metabolic enzyme deficiencies. Although mice with disrupted SCID-causing genes have provided important insights into the human disease, not all the SCID mice have phenotypes that resemble those in human SCID patients. In humans, most SCID patients are reported to have impaired cytokine-mediated signalling in immune cells. IL2RG is a key component of the immune system, which is associated with the development of X-linked SCID in humans. Despite some phenotypic characterisations and functional studies being performed in SCID animals, little is known about the molecular basis of the different phenotypes of SCID in mouse and pig. In the present experiment, we generated monoallelic IL2RG (mIL2RG+/Δ69-368) KO pigs and investigated patterns of gene expression during their immune development in order to further explore our understanding of immune responses in X-linked SCID.
2016-09-27 | GSE77581 | GEO
Project description:Severe combined immunodeficiency (SCID) occurs in various species, including humans, at a frequency as high as one per 50,000 live births. Generally, SCID can be classified according to the cause of the immunodeficiency, and it includes impaired cytokine-mediated signalling, defective V(D)J recombination, impaired pre-T-cell receptor signalling, and metabolic enzyme deficiencies. Although mice with disrupted SCID-causing genes have provided important insights into the human disease, not all the SCID mice have phenotypes that resemble those in human SCID patients. In humans, most SCID patients are reported to have impaired cytokine-mediated signalling in immune cells. IL2RG is a key component of the immune system, which is associated with the development of X-linked SCID in humans. Despite some phenotypic characterisations and functional studies being performed in SCID animals, little is known about the molecular basis of the different phenotypes of SCID in mouse and pig. In the present experiment, we generated monoallelic IL2RG (mIL2RG+/Δ69-368) KO pigs and investigated patterns of gene expression during their immune development in order to further explore our understanding of immune responses in X-linked SCID.
2016-09-27 | GSE77580 | GEO
Project description:Severe combined immunodeficiency (SCID) occurs in various species, including humans, at a frequency as high as one per 50,000 live births. Generally, SCID can be classified according to the cause of the immunodeficiency, and it includes impaired cytokine-mediated signalling, defective V(D)J recombination, impaired pre-T-cell receptor signalling, and metabolic enzyme deficiencies. Although mice with disrupted SCID-causing genes have provided important insights into the human disease, not all the SCID mice have phenotypes that resemble those in human SCID patients. In humans, most SCID patients are reported to have impaired cytokine-mediated signalling in immune cells. IL2RG is a key component of the immune system, which is associated with the development of X-linked SCID in humans. Despite some phenotypic characterisations and functional studies being performed in SCID animals, little is known about the molecular basis of the different phenotypes of SCID in mouse and pig. In the present experiment, we generated monoallelic IL2RG (mIL2RG+/Δ69-368) KO pigs and investigated patterns of gene expression during their immune development in order to further explore our understanding of immune responses in X-linked SCID.
2016-09-27 | GSE77579 | GEO
Project description:Gene expression profile of the RAG2 gene depletion on spleen, thymus, and LN of mouse
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Project description:Gene expression profile of the RAG2/pfp gene depletion on mouse spleen, lymph node (LN), and thymus
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Project description:Pigs with severe combined immunodeficiency (SCID) may provide useful models for regenerative medicine, xenotransplantation, and tumor development and will aid in developing therapies for human SCID patients. Using a reporter-guided transcription activator-like effector nuclease (TALEN) system, we generated targeted modifications of recombination activating gene (RAG) 2 in somatic cells at high efficiency, including some that affected both alleles. Somatic-cell nuclear transfer performed with the mutated cells produced pigs with RAG2 mutations without integrated exogenous DNA. Biallelically modified pigs either lacked a thymus or had one that was underdeveloped. Their splenic white pulp lacked B and T cells. Under a conventional housing environment, the biallelic RAG2 mutants manifested a failure to thrive phenotype, with signs of inflammation and apoptosis in the spleen compared with age-matched wild-type animals by the time they were 4 wk of age. Pigs raised in a clean environment were healthier and, following injection of human induced pluripotent stem cells (iPSCs), quickly developed mature teratomas representing all three germ layers. The pigs also tolerated grafts of allogeneic porcine trophoblast stem cells. These SCID pigs should have a variety of uses in transplantation biology.
2017-08-31 | GSE97502 | GEO
Project description:Gene expression profile of the IL2RG gene depletion in pig thymus
| PRJNA310861 | ENA
Project description:Pigs with severe combined immunodeficiency (SCID) may provide useful models for regenerative medicine, xenotransplantation, and tumor development and will aid in developing therapies for human SCID patients. Using a reporter-guided transcription activator-like effector nuclease (TALEN) system, we generated targeted modifications of recombination activating gene (RAG) 2 in somatic cells at high efficiency, including some that affected both alleles. Somatic-cell nuclear transfer performed with the mutated cells produced pigs with RAG2 mutations without integrated exogenous DNA. Biallelically modified pigs either lacked a thymus or had one that was underdeveloped. Their splenic white pulp lacked B and T cells. Under a conventional housing environment, the biallelic RAG2 mutants manifested a failure to thrive phenotype, with signs of inflammation and apoptosis in the spleen compared with age-matched wild-type animals by the time they were 4 wk of age. Pigs raised in a clean environment were healthier and, following injection of human induced pluripotent stem cells (iPSCs), quickly developed mature teratomas representing all three germ layers. The pigs also tolerated grafts of allogeneic porcine trophoblast stem cells. These SCID pigs should have a variety of uses in transplantation biology.
2017-08-31 | GSE97501 | GEO
Project description:Gene expression profile of the IL2RG gene depletion in pig spleen
| PRJNA310860 | ENA