At Ace Therapeutics, we specialize in developing disease models to advance the understanding and treatment of biliary atresia (BA). With our expertise in disease modeling, we offer tailored biliary atresia disease models that accurately recapitulate the pathological features observed in patients, including inflammation, fibrosis, and bile duct obstruction. These models serve as powerful tools for studying disease mechanisms, evaluating potential treatments, and accelerating progress in biliary atresia research.
What Is Biliary Atresia?
Biliary atresia is a rare congenital disorder in which the bile ducts inside or outside the liver are blocked or absent, preventing bile from draining from the liver to the gallbladder. This obstruction causes bile to build up in the liver, leading to liver damage, fibrosis, and cirrhosis. Pathologically, it results in progressive inflammation and scarring of the liver tissue. Early symptoms include jaundice and pale stools. Drug treatments for biliary atresia typically involve the use of medications to manage symptoms, promote bile flow, and mitigate liver damage. These include ursodeoxycholic acid (UDCA), corticosteroids, immunosuppressive agents, and vitamins.
Figure 1. Immunohistochemical staining results and animal models of biliary atresia (Pal N., et al., 2022)
How Do We Customize Biliary Atresia Models?
- Custom In Vitro Disease Model of Biliary Atresia
Cell Culture Systems
We use primary human hepatocytes and bile duct epithelial cells to create organoid cultures that mimic the cellular environment of the liver. These organoids provide a powerful platform for conducting detailed studies on bile duct formation and pathology, enabling a deeper understanding of disease mechanisms and potential therapeutic targets.
Custom Cell Lines
Our expert team harnesses the power of CRISPR technology to develop customized cell lines with specific mutations in key genes associated with biliary atresia. By precisely editing the genes involved in biliary atresia, we help researchers investigate the molecular mechanisms that drive the disease, paving the way for targeted therapies and advances in the field.
- Custom Animal Models of Biliary Atresia
Ace Therapeutics offers customized animal models for BA research. Our services allow researchers to select specific animal species, modeling methods, and disease features to best suit their research objectives.
|
Modeling Methods |
Model Types |
Details of Our Biliary Atresia Models |
| Surgical Models |
Bile duct ligation (BDL) models |
A classic model |
| Transplantation models |
Transplantation of bile duct segments from mice of different ages into adult mice results in fibrosclerosis and histological changes resembling BA. |
| Virus-Induced Models |
Rhesus rotavirus (RRV)-induced models |
RRV-induced BA mice exhibit features similar to human BA, which are the most widely used models. |
| CMV-induced models |
A perinatal CMV-induced hepatobiliary system injury model in guinea pigs shows biliary tropism and inflammatory injury, potentially relevant to the pathogenesis of BA. |
| Rotavirus-induced models |
Oral or intraperitoneal administration of group A rotavirus to newborn mice causes extrahepatic biliary obstruction and histological changes resembling human BA. |
| Toxin/Drug-Induced Models |
PMA-induced models |
Infusing phorbol myristate acetate (PMA) into the gallbladder of hamsters induces peripheral neutrophil activation and inflammation. |
| Biliatresone-induced models |
Biliatresone, an isoflavonoid found in certain plants, causes biliary damage in lambs and calves. We can develop both zebrafish and mouse models that show dose-dependent effects on biliary morphology. |
| Genetic Models |
Inv mutant mouse model |
Inversin (inv) mutant mice exhibit situs inversus and severe jaundice, with intrahepatic bile duct changes resembling DPM seen in some human BA cases. |
| Sox17 haploinsufficiency mouse model |
Sox17+/− mice show defects in epithelial maturation and congenital biliary atresia in extrahepatic ducts. |
| Pkhd1 mutant mouse model |
NOD.Abd3 mice with aberrant Pkhd1 expression exhibit lymphocytic infiltration and biliary duct epithelial hyperplasia, potentially relevant to BA pathogenesis. |
Ace Therapeutics offers customized disease models of biliary atresia, tailored to your specific research needs. Whether you're investigating specific pathogenic pathways, evaluating novel therapies, or exploring the underlying mechanisms of BA, we can help you achieve your research goals. Contact us today to discuss your specific requirements.
Reference
- Pal N., et al. Biliary atresia animal models: Is the needle in a haystack? Int J Mol Sci. 2022, 23(14):7838.
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