B6-IgA KO Mice

Catalog Number: C001394

Strain Name: C57BL/6JCya-Ighaem1/Cya

Genetic Background: C57BL/6JCya

Reproduction: Homozygote x Homozygote

 

Strain Description

Immunoglobulin A (IgA) is the second most abundant immunoglobulin in serum, comprising 10–20% of serum immunoglobulins, following IgG. IgA is primarily produced by the mucosal tissues of the digestive, respiratory, and urogenital systems, where mucosal-associated lymphoid tissues generate IgA to counteract pathogen invasion. Additionally, IgA is present in saliva, tears, and breast milk [1]. In the human body, IgA can be classified into serum IgA and secretory IgA based on its location. Serum IgA exhibits relatively weak immune functions [2], whereas secretory IgA is a critical component of the mucosal defense system. It is widely distributed in breast milk, saliva, and mucosal secretions of the gastrointestinal, respiratory, and urogenital tracts, playing an essential role in inhibiting microbial adhesion to respiratory epithelium and reducing viral replication. IgA possesses antibody activity against certain viruses, bacteria, and general antigens, serving as the first line of defense against pathogen invasion [3].

Selective immunoglobulin A (IgA) deficiency is a systemic immunological disorder caused by a primary immunodeficiency characterized by the absence of detectable IgA in the blood and secretions, while other immunoglobulin types remain unaffected. The function of T lymphocytes, phagocytes, and the complement system in these patients is also preserved. Approximately half of the patients with selective IgA deficiency are susceptible to recurrent infections, such as otitis media, sinusitis, bronchitis, and pneumonia [4]. Moreover, these patients exhibit a significantly increased prevalence of autoimmune diseases, including rheumatoid arthritis, systemic lupus erythematosus, and immune thrombocytopenic purpura, as well as allergic conditions such as asthma [4-5].

The B6-IgA KO mouse is an IgA-deficient model developed by knocking out the mouse Igha gene, which encodes IgA, in mice. This mouse model provides a valuable tool for studying diseases associated with selective IgA deficiency.

Strain Strategy

The Igha gene is located on chromosome 12 in mice, and exon 1~4 were knocked out using gene editing technology.



Application

  • Study of selective immunoglobulin A (IgA) deficiency;
  • Research on autoimmune and allergic diseases related to IgA deficiency;
  • Investigation of the mechanisms and functions of immunoglobulin A (IgA).

 

Validation Data

1. Serum IgA Content Analysis


Figure 1. Serum IgA content in eight-week-old B6-IgA KO mice compared to wild-type mice. Peripheral blood was collected from B6-IgA KO mice (IgA-KO) and wild-type mice (WT) to isolate serum. Serum IgA levels were measured using ELISA. The results demonstrated a significant difference in serum IgA levels between B6-IgA KO mice and wild-type mice, with B6-IgA KO mice showing almost no IgA expression in their serum.

Conclusion

The Igha gene was knocked out in B6-IgA KO mouse using gene editing technology to establish an IgA-deficient mouse model. B6-IgA KO mouse was evaluated through serum ELISA assays. The results showed that, compared to wild-type control mice, the serum IgA levels in B6-IgA KO mice were significantly reduced, with almost no detectable expression, confirming the successful establishment of the model. This model can be used in subsequent studies related to diseases based on IgA deficiency, providing a robust and effective tool for human disease research.

 

References
[1]Pabst O. New concepts in the generation and functions of IgA. Nat Rev Immunol. 2012 Dec;12(12):821-32.
[2]de Fays C, Carlier FM, Gohy S, Pilette C. Secretory Immunoglobulin A Immunity in Chronic Obstructive Respiratory Diseases. Cells. 2022 Apr 13;11(8):1324.
[3]Corthésy B. Role of secretory immunoglobulin A and secretory component in the protection of mucosal surfaces. Future Microbiol. 2010 May;5(5):817-29.
[4]Yazdani R, Azizi G, Abolhassani H, Aghamohammadi A. Selective IgA Deficiency: Epidemiology, Pathogenesis, Clinical Phenotype, Diagnosis, Prognosis and Management. Scand J Immunol. 2017 Jan;85(1):3-12.
[5]Hiemstra PS. Immunoglobulin A in asthma: friend or foe? Eur Respir J. 1998 Sep;12(3):517-8.