C3 Glomerulonephritis (C3GN)

With unwavering focus towards developing novel therapies for C3 Glomerulonephritis (C3GN), Protheragen is dedicated to advancing research on kidney diseases. From target validation to clinical milestone achievement, we help our partners through IND-enabling studies and provide comprehensive solutions.

Introduction to C3 Glomerulonephritis

C3 Glomerulonephritis is a rare disease characterized by an unstable alternative pathway of AP complement with dominant C3 deposition and leads to dysregulation. Autoantibodies like C3 nephritic factors, CFH and CFI mutations disrupt homeostasis and result in inflammation of glomeruli, leading to proteinuria and progressive CKD. C3GN affects about 1-2 people per million and increases the risk of ESKD within a decade, which increases the hunt for targeted therapeutic solutions.

Pathogenesis of C3 Glomerulonephritis

C3 Glomerulonephritis stems from the inability to control the alternative pathway of the complement system, usually due to genetic mutations. This leads to uncontrolled activation of C3 which deposits C3 fragments in the glomeruli and initiates inflammation. This causes injury to glomeruli which results in kidney dysfunction.

Fig.1 Points of action of the different potential causative factors of C3G in the complement cascade. (Kaartinen et al., 2019)

Therapeutics Development for C3 Glomerulonephritis

Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a treatment plan recommendation. For guidance on treatment options, please visit a regular hospital.

Our Services

Protheragen is a comprehensive preclinical service provider dedicated to the discovery and development of novel therapeutics for C3 Glomerulonephritis. Our extensive therapeutic development services incorporate the use of sophisticated disease models that effectively capture important features of this inherited and progressive kidney disorder.

Therapeutic Development Platforms of C3 Glomerulonephritis

• Small Molecule Drug
• Cell Therapy
• Gene Therapy

• Therapeutic Antibody
• Therapeutic Peptide
• Therapeutic Protein

Disease Models Development for C3 Glomerulonephritis

Protheragen offers innovative and comprehensive disease models to advance preclinical research into C3GN. Our platform integrates advanced cell-based models, kidney organoids, and animal models to effectively capture the genetic, immunological, and pathological signatures of complement dysregulation and their impact on renal health and glomerular injury.

Cell-based & Organoid Models

• Human glomerular endothelial cell models
• Podocyte injury models with complement exposure
• Mesangial cell proliferation models
• Complement component-expressing renal cell lines
• iPSC-derived kidney organoids

Animal Models Development

• Complement Factor H (Cfh) knockout/mutant mouse models
• C3 transgenic mouse models with complement dysregulation
• Cfi (Complement Factor I) knockout mouse models
• Zebrafish complement pathway mutant lines

Protheragen's integrated preclinical development solutions are specifically designed for research in complex renal and immunological disorders like C3GN. We specialize in custom disease model development, pharmacokinetics, and drug safety evaluation, enabling us to support your investigation from target validation to IND-enabling studies.

Contact us today to accelerate your C3GN research with our end-to-end solutions.

References

1. Lanktree, M. B., et al. "Insights into C3 Glomerulonephritis from Genetic Studies." Clin J Am Soc Nephrol 16.5 (2021): 790-99. Print.
2. Reiterova, J., and V. Tesar. "C3 Glomerulonephritis: From Pathophysiology of Cystogenesis to Advances in the Treatment." Int J Mol Sci 23.6 (2022).

All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.