ArticleLaboratory investigation; a journal of technical methods and pathology2020
The synthetic toxin biliatresone causes biliary atresia in mice.
Article in Laboratory investigation; a journal of technical methods and pathology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 31 citations in OpenAlex.
- Dual Functions and Therapeutic Potential of FZD6 in Biliary Atresia.Digestive diseases and sciences · 2025Article
- Review
- Single-cell transcription reveals hepatocyte-to-cholangiocyte reprogramming and biliary gene profile in biliary atresia.Hepatology communications · 2025Article
- Biliary atresia susceptibility gene EFEMP1 regulates extrahepatic bile duct elastic fiber formation and mechanics.JHEP reports : innovation in hepatology · 2025Article
- Microcystin-RR is a biliary toxin selective for neonatal extrahepatic cholangiocytes.JHEP reports : innovation in hepatology · 2025Article
- Generation of human induced pluripotent stem cell lines derived from patients of cystic biliary atresia.Human cell · 2024Article
- Mechanism of biliary atresia caused by T follicular helper cells-induced immune injury.BMC pediatrics · 2024Article
- Biliary atresia.Nature reviews. Disease primers · 2024Review
- Article
- Biliary atresia: the role of gut microbiome, and microbial metabolites.Frontiers in cellular and infection microbiology · 2024Review
- Article
- Review
- Biliatresone induces cholangiopathy in C57BL/6J neonates.Scientific reports · 2023Article
- Biliatresone: progress in biliary atresia study.World journal of pediatrics : WJP · 2023Review
- Incidence of Isolated Biliary Atresia during the COVID Lockdown in Europe: Results from a Collaborative Project by RARE-Liver.Journal of clinical medicine · 2023Article
- Immune-mediated cholangiopathies in children: the need to better understand the pathophysiology for finding the future possible treatment targets.Frontiers in immunology · 2023Review
- Biliary Atresia in Children: Update on Disease Mechanism, Therapies, and Patient Outcomes.Clinics in liver disease · 2022Review
- Biliary Atresia Animal Models: Is the Needle in a Haystack?International journal of molecular sciences · 2022Review
- Biliary Atresia: A Complex Hepatobiliary Disease with Variable Gene Involvement, Diagnostic Procedures, and Prognosis.Diagnostics (Basel, Switzerland) · 2022Review
- Biliary Atresia - emerging diagnostic and therapy opportunities.EBioMedicine · 2021Review
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Exposure to environmental toxins may be responsible for biliary atresia. The focus of this study was to investigate the effect of biliatresone on the development of the hepatobiliary system in mice. We successfully synthesized biliatresone with a purity of 98% and confirmed its biliary toxicity. Exposure to high doses of biliatresone caused abortion or death in pregnant mice. Neonatal mice injected with biliatresone developed clinical signs of biliary obstruction, and dysplasia or the absence of extrahepatic biliary tract lumen, which confirmed the occurrence of biliary atresia. In the portal tract of biliary atresia mice, signs of infiltration of inflammatory cells and liver fibrosis were observed. The signature of extrahepatic biliary gene expression in these mice mainly involved the cell adhesion process, and hepatic RNA-seq was highly linked to transcriptional evidence of oxidative stress. When compared with the control group, hepatic glutathione levels were markedly reduced after biliatresone injection. Taken together, these data confirm that biliatresone causes severe developmental abnormalities of the hepatobiliary system in mice. Furthermore, decreased levels of glutathione may play a mechanistic role in the pathogenesis of liver fibrosis in biliatresone-induced experimental biliary atresia.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.