B6-hIL2RG Mouse
Product Name
B6-hIL2RG Mouse
Product ID
C001801
Strain Name
C57BL/6NCya-Il2rgtm1(hIL2RG)/Cya
Backgroud
C57BL/6NCya
Status
When using this mouse strain in a publication, please cite “B6-hIL2RG Mouse (Catalog C001801) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Gene Name
IL2RG
Gene Alias
P64, CIDX, IMD4, CD132, SCIDX, IL-2RG, SCIDX1
NCBI ID
Chromosome
Chr X (Human)
MGI ID
Datasheet
Strain Description
The IL2RG (Interleukin 2 Receptor Subunit Gamma) gene, located on the X chromosome, provides instructions for making the common gamma chain protein (also known as CD132 or γc). This protein is a critical signaling component shared by the receptors for several interleukins, including IL-2, IL-4, IL-7, IL-9, IL-15, and IL-21. IL2RG is widely expressed, particularly in lymphoid tissues like the lymph nodes, appendix, thymus, and spleen, as well as in immature blood-forming cells in the bone marrow, and also detected in other tissues such as skin and intestine [1-2]. The common gamma chain is essential for the development, proliferation, and function of various immune cells, including T cells, B cells, and natural killer (NK) cells, by facilitating intracellular signaling pathways such as JAK/STAT, MAPK, and PI3K/AKT, which are crucial for immune system homeostasis [1-2]. Mutations in the IL2RG gene are primarily associated with X-linked severe combined immunodeficiency (X-SCID), a severe inherited disorder causing profound defects in both cellular and humoral immunity, typically affecting males and leading to recurrent, life-threatening infections [3-4]. Less severe "hypomorphic" mutations can cause X-linked combined immunodeficiency (XCID), and IL2RG dysregulation has also been implicated in certain cancers and autoimmune conditions [1].
The B6-hIL2RG mouse is a humanized model, constructed by replacing the coding sequences of the endogenous mouse Il2rg gene with the coding sequences of the human IL2RG gene. B6-hIL2RG mice can be used for research into the pathogenesis of X-linked severe combined immunodeficiency (X-SCID), certain autoimmune diseases, and hematological malignancies, as well as for the screening, development, and safety evaluation of IL2RG-targeted drugs.
Reference
Winer H, Rodrigues GOL, Hixon JA, Aiello FB, Hsu TC, Wachter BT, Li W, Durum SK. IL-7: Comprehensive review. Cytokine. 2022 Dec;160:156049.
Lin JX, Leonard WJ. The Common Cytokine Receptor γ Chain Family of Cytokines. Cold Spring Harb Perspect Biol. 2018 Sep 4;10(9):a028449.
Puck JM, Pepper AE, Henthorn PS, Candotti F, Isakov J, Whitwam T, Conley ME, Fischer RE, Rosenblatt HM, Small TN, Buckley RH. Mutation analysis of IL2RG in human X-linked severe combined immunodeficiency. Blood. 1997 Mar 15;89(6):1968-77.
Lim CK, Abolhassani H, Appelberg SK, Sundin M, Hammarström L. IL2RG hypomorphic mutation: identification of a novel pathogenic mutation in exon 8 and a review of the literature. Allergy Asthma Clin Immunol. 2019 Jan 5;15:2.
Strain Strategy
The sequences from the ATG start codon to the TGA stop codon of the endogenous mouse Il2rg gene will be replaced with the sequences from the ATG start codon to the TGA stop codon of the human IL2RG gene.
Figure 1. Gene editing strategy of B6-hIL2RG Mice.
Application Area
IL2RG-targeted drug screening, development, and evaluation;
Research on the pathological mechanisms and therapeutic approaches of X-linked severe combined immunodeficiency (X-SCID);
Research on the pathological mechanisms and therapeutic approaches of certain autoimmune diseases;
Research on the pathological mechanisms and therapeutic approaches of hematological malignancies.
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