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Rabbit Anti-IL2RG Recombinant Antibody (005) (CBMAB-C1528-LY)


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Summary

Host Animal
Rabbit
Specificity
Mouse
Clone
005
Antibody Isotype
IgG
Application
ELISA, ELISA(Det)

Basic Information

Immunogen
Recombinant Mouse CD132 / IL2RG protein
Specificity
Mouse
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Interleukin 2 Receptor Subunit Gamma
Introduction
The protein encoded by this gene is an important signaling component of many interleukin receptors, including those of interleukin -2, -4, -7 and -21, and is thus referred to as the common gamma chain. Mutations in this gene cause X-linked severe combined immunodeficiency (XSCID), as well as X-linked combined immunodeficiency (XCID), a less severe immunodeficiency disorder. [provided by RefSeq, Mar 2010]
Entrez Gene ID
16186
UniProt ID
P34902
Function
Common subunit for the receptors for a variety of interleukins. Probably in association with IL15RA, involved in the stimulation of neutrophil phagocytosis by IL15 (PubMed:15123770).
Biological Process
Cytokine-mediated signaling pathwayManual Assertion Based On ExperimentIBA:GO_Central
Immune responseManual Assertion Based On ExperimentTAS:ProtInc
Interleukin-15-mediated signaling pathwayManual Assertion Based On ExperimentIMP:UniProtKB
Interleukin-2-mediated signaling pathwayIEA:GOC
Interleukin-4-mediated signaling pathwayIEA:GOC
Interleukin-7-mediated signaling pathwayIEA:GOC
Positive regulation of phagocytosisManual Assertion Based On ExperimentIMP:UniProtKB
Signal transduction1 PublicationNAS:ProtInc
Cellular Location
Cell membrane; Cell surface
Involvement in disease
Severe combined immunodeficiency X-linked T-cell-negative/B-cell-positive/NK-cell-negative (XSCID):
A form of severe combined immunodeficiency (SCID), a genetically and clinically heterogeneous group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. Patients present in infancy recurrent, persistent infections by opportunistic organisms. The common characteristic of all types of SCID is absence of T-cell-mediated cellular immunity due to a defect in T-cell development.
X-linked combined immunodeficiency (XCID):
Less severe form of X-linked immunodeficiency with a less severe degree of deficiency in cellular and humoral immunity than that seen in XSCID.
Topology
Extracellular: 23-262
Helical: 263-283
Cytoplasmic: 284-369
More Infomation

Tuovinen, E. A., Pöysti, S., Hamdan, F., Le, K. M., Keskitalo, S., Turunen, T., ... & Grönholm, J. (2023). Characterization of expanded gamma delta T cells from atypical X-SCID patient reveals preserved function and IL2RG-mediated signaling. Journal of clinical immunology, 43(2), 358-370.

Zheng, X., Huang, C., Lin, Y., Han, B., Chen, Y., Li, C., ... & Yan, S. (2023). Generation of inactivated IL2RG and RAG1 monkeys with severe combined immunodeficiency using base editing. Signal Transduction and Targeted Therapy, 8(1), 327.

Goncalves, P., Doisne, J. M., Eri, T., Charbit, B., Bondet, V., Posseme, C., ... & Milieu Intérieur Consortium. (2022). Defects in mucosal immunity and nasopharyngeal dysbiosis in HSC-transplanted SCID patients with IL2RG/JAK3 deficiency. Blood, The Journal of the American Society of Hematology, 139(17), 2585-2600.

Yue, X., Petersen, F., Shu, Y., Kasper, B., Magatsin, J. D. T., Ahmadi, M., ... & Riemekasten, G. (2021). Transfer of PBMC from SSc patients induces autoantibodies and systemic inflammation in Rag2-/-/IL2rg-/-mice. Frontiers in immunology, 12, 677970.

Hou, Y., Gratz, H. P., Ureña-Bailén, G., Gratz, P. G., Schilbach-Stückle, K., Renno, T., ... & Mezger, M. (2021). Somatic Reversion of a Novel IL2RG Mutation Resulting in Atypical X-Linked Combined Immunodeficiency. Genes, 13(1), 35.

Ren, J., Yu, D., Fu, R., An, P., Sun, R., Wang, Z., ... & Hu, Z. (2020). IL2RG‐deficient minipigs generated via CRISPR/Cas9 technology support the growth of human melanoma‐derived tumours. Cell Proliferation, 53(10), e12863.

Tuovinen, E. A., Grönholm, J., Öhman, T., Pöysti, S., Toivonen, R., Kreutzman, A., ... & Seppänen, M. R. (2020). Novel hemizygous IL2RG p.(Pro58Ser) mutation impairs IL-2 receptor complex expression on lymphocytes causing X-linked combined immunodeficiency. Journal of Clinical Immunology, 40, 503-514.

Boettcher, A. N., Li, Y., Ahrens, A. P., Kiupel, M., Byrne, K. A., Loving, C. L., ... & Tuggle, C. K. (2020). Novel engraftment and T cell differentiation of human hematopoietic cells in ART−/− IL2RG−/Y SCID pigs. Frontiers in Immunology, 11, 100.

Lim, C. K., Abolhassani, H., Appelberg, S. K., Sundin, M., & Hammarström, L. (2019). IL2RG hypomorphic mutation: identification of a novel pathogenic mutation in exon 8 and a review of the literature. Allergy, Asthma & Clinical Immunology, 15, 1-8.

Miggelbrink, A. M., Logan, B. R., Buckley, R. H., Parrott, R. E., Dvorak, C. C., Kapoor, N., ... & Pai, S. Y. (2018). B-cell differentiation and IL-21 response in IL2RG/JAK3 SCID patients after hematopoietic stem cell transplantation. Blood, The Journal of the American Society of Hematology, 131(26), 2967-2977.

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For research use only. Not intended for any clinical use.

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