B6-hIL6RA Mouse
Product Name
B6-hIL6RA Mouse
Product ID
C001606
Strain Name
C57BL/6NCya-Il6raem1(hIL6RA)/Cya
Backgroud
C57BL/6NCya
Status
When using this mouse strain in a publication, please cite “B6-hIL6RA Mouse (Catalog C001606) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Gene Name
IL6R
Gene Alias
IL6Q, gp80, CD126, HIES5, IL-6R, IL6RA, IL6RQ, IL-1Ra, IL-6RA, IL6QTL, IL-6R-1
NCBI ID
Chromosome
Chr 1 (Human)
MGI ID
Datasheet
Strain Description
The IL6RA (IL6R, also known as CD126) gene encodes a subunit of the interleukin-6 (IL-6) receptor complex. The IL-6 receptor is a protein complex composed of the IL6RA protein and the interleukin-6 signal transducer (IL6ST/GP130/IL6-beta). This receptor subunit is shared by many other cytokines. The expression of IL-6R is primarily restricted to hepatocytes, leukocytes, and megakaryocytes. Upon binding to its receptor IL-6Rα, IL-6 interacts with two GP130 molecules to form a hexameric complex in a 2:2:2 configuration. Once the IL-6 receptor complex is activated, multiple downstream events allow IL-6 to mediate its diverse effects. These include the pathways of the GTPase Ras and its effector Raf, the mitogen-activated protein kinase cascade that controls cellular proliferation and differentiation, and the pathways involving tyrosine kinases of the Jak family and transcription factors of the STAT family [1]. IL-6 receptor defects can lead to immunodeficiency and atopy. Patients with loss-of-function variants in IL-6R present with an autosomal recessive disorder characterized by recurrent Haemophilus chest infections, staphylococcal skin abscesses, atopic dermatitis, elevated IgE levels, eosinophilia, and absent acute phase responses [2]. Research has shown that the IL-6 pathway is crucial for maintaining homeostasis and is involved in the dysregulation seen in many diseases. Antibody drugs targeting the IL-6/IL-6 receptor signaling pathway have become innovative therapies for autoimmune diseases and cancers [3].
The B6-hIL6RA mouse is an Il6ra gene humanized model, in which the coding sequence (CDS) of the human IL6R gene is inserted into the endogenous Il6ra gene sequence of mice. This model can be used in researching autoimmune diseases, inflammation-related diseases, cancer, and infectious diseases. It is also useful for the development, screening, and evaluation of IL6RA-targeted drugs.
Reference
Hunter CA, Jones SA. IL-6 as a keystone cytokine in health and disease. Nat Immunol. 2015 May;16(5):448-57.
Chen YH, Spencer S, Laurence A, Thaventhiran JE, Uhlig HH. Inborn errors of IL-6 family cytokine responses. Curr Opin Immunol. 2021 Oct;72:135-145.
The application and development of IL-6 pathway inhibitors[J]. International Journal of Biologicals, 2020, 43(5): 239-242.
Strain Strategy
The partial exon 1 coding region of mouse Il6ra was replaced with the Kozak-IL6R Chimera CDS-3’UTR of Mouse Il6ra-WPRE-BGH pA cassette.
*IL6R Chimera CDS: Signal Peptide and Extracellular Domain of Human IL6R +Transmembrane and Cytoplasmic Domain of Mouse Il6ra
Figure 1. Gene editing strategy of B6-hIL6RA mice.
Application Area
Research on autoimmune diseases, inflammation-related diseases, cancer, and infectious diseases；
Preclinical studies on the development, screening, and evaluation of IL6RA-targeted drugs.
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