Immunoglobulin A Nephropathy (IgAN)
Immunoglobulin A nephropathy (IgAN) also known as Berger's disease, is a chronic kidney disease characterized by the deposition of immunoglobulin A (IgA) in the glomeruli of the kidneys. Our company with a team of experts and state-of-the-art facilities ensures that researchers have access to the resources and support necessary for advancing their research.
Overview of IgAN
IgAN is a primary glomerular disease with an annual incidence of 2 to 10 per 100,000 individuals. The primary abnormality in IgAN is the production of abnormal IgA1 antibodies by the immune system. These IgA1 antibodies have an aberrant structure, leading to their deposition in the glomeruli, causing inflammation and damage to the kidneys, leading to problems with their function.
Fig.1 Pathological Characteristics of IgAN. (Zhang, Y. Z., et al., 2021)Pathogenesis of IgAN
Genetic factors, infections, and environmental factors play a role in the development of IgAN. The deposited IgA1 antibodies trigger an inflammatory response, causing damage to the glomeruli and leading to the characteristic features of IgAN, including glomerular inflammation and scarring. Various factors, such as infections, abnormal glycosylation of IgA1, and genetic predisposition, contribute to the development and progression of the disease.
Fig.2 The multi-hit hypothesis for pathogenesis of IgAN. (Rajasekaran, A., et al., 2021)Therapeutics Development of IgAN
| Names | Therapeutics Types | Mechanism of Action | Research Phase |
|---|---|---|---|
| TRF Budesonide | Small molecule drug | Corticosteroid formulation acts on distal ileum targeting B-cells in mucosal lymphoid tissue | Phase III trials |
| Hydroxychloroquine | Small molecule drug | Immunomodulator, inhibits mucosal and intrarenal Toll-like receptor signaling | Phase II trials |
| Bortezomib | Small molecule drug | Semi-selective plasma cell proteasome inhibitor | Phase IV trials |
| Fostamatinib | Small molecule drug | Oral spleen tyrosine kinase inhibitor | Phase II trials |
| VIS649 | Antibody | Monoclonal antibody against APRIL | Phase II trials |
| Narsoplimab | Antibody | Monoclonal antibody against MASP-2 | Phase II trials |
| Cemdisiran | Small-interfering RNA | Small-interfering RNA inhibits synthesis of C5 | Phase II trials |
Our Services
Our company stands out in the field of rare disease therapy and development with our unwavering commitment to providing comprehensive services for researchers. Through our animal model and therapeutic development platform, we can meet the specific needs of researchers working in this area.
Platforms of IgAN Therapy Development
Animal Models of IgAN
Animal models play a crucial role in understanding the pathogenesis of diseases like IgAN and in developing potential therapeutic interventions. Our company provides a variety of animal models of IgAN to enhance your understanding of IgAN, and evaluate the efficacy of potential therapeutics.
Chemical-induced animal models involve the administration of specific chemicals to induce renal injury and glomerular deposition of IgA, which provide a controlled environment for studying specific aspects of IgAN pathogenesis.
Optional Models: Dextran-induced model; IgA-albumin immune complexes-induced model, etc.
Genetic engineering models of IgAN utilize techniques such as transgenic or knockout technology to modify specific genes involved in disease pathogenesis to generate animals with characteristics similar to human IgAN individuals.
Optional Models: Uteroglobin-deficient model; CD89-overexpressing model; CD37-deficient model; LIGHT-overexpressing model, etc.
With a deep understanding of the complexities and challenges associated with rare diseases, our company offers comprehensive services through our specialized expertise and cutting-edge technologies, including pharmacokinetic research and drug safety evaluation.
If you are interested in learning more about our services and how we can support your research endeavors, please do not hesitate to reach out to us for further information.
References
- Rajasekaran, Arun et al. "IgA Nephropathy: An Interesting Autoimmune Kidney Disease." The American journal of the medical sciences 361,2 (2021): 176-194.
- Zhang, Yong-Zhe et al. "Immunoglobulin a nephropathy as the first clinical presentation of Wilson disease: a case report and literature review." BMC gastroenterology 21.1 (2021): 384.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.