biliary atresia/glutathione

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alpha-Glutathione-s-transferase as a new sensitive marker of hepatocellular damage in biliary atresia.

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Identifikohuni Regjistrohu

Early identification of patients likely to deteriorate post-hepatic portoenterostomy for biliary atresia (BA) would be beneficial. alpha-Glutathione-s-transferase (alpha-GST) is a serologic marker of reactive hepatocellular damage because of its low molecular weight, uniform hepatic distribution,

Extrahepatic cholangiocyte obstruction is mediated by decreased glutathione, Wnt and Notch signaling pathways in a toxic model of biliary atresia.

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Identifikohuni Regjistrohu

Biliary atresia is a neonatal liver disease with extrahepatic bile duct obstruction and progressive liver fibrosis. The etiology and pathogenesis of the disease are unknown. We previously identified a plant toxin, biliatresone, responsible for biliary atresia in naturally-occurring animal models,

Reactivity of Biliatresone, a Natural Biliary Toxin, with Glutathione, Histamine, and Amino Acids.

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Identifikohuni Regjistrohu

In our previous work, we identified a natural toxin, biliatresone, from Dysphania glomulifera and D. littoralis, endemic plants associated with outbreaks of biliary atresia in Australian neonatal livestock. Biliatresone is a very rare isoflavonoid with an α-methylene ketone between two phenyls,

Glutathione antioxidant pathway activity and reserve determine toxicity and specificity of the biliary toxin biliatresone in zebrafish.

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Identifikohuni Regjistrohu

Biliatresone is an electrophilic isoflavone isolated from Dysphania species plants that has been causatively linked to naturally occurring outbreaks of a biliary atresia (BA)-like disease in livestock. Biliatresone has selective toxicity for extrahepatic cholangiocytes (EHCs) in zebrafish larvae. To

Selenium and glutathione peroxidase status in paediatric health and gastrointestinal disease.

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Identifikohuni Regjistrohu

To examine the effect of paediatric GI disease on selenium concentration in plasma and glutathione peroxidase activity in plasma and red cells, results in children with Crohn's disease (n = 39), cystic fibrosis (n = 14), intractable diarrhoea (n = 13), and biliary atresia (n = 10) were compared with

Correlation between hepatic oxidative damage and clinical severity and mitochondrial gene sequencing results in biliary atresia.

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Identifikohuni Regjistrohu

To assess the level of hepatic oxidative damage and its correlation with clinical severity in biliary atresia (BA), and to understand BA mitochondrial gene sequencing.Forty-eight BA patients and 28 control subjects (20 hepatoblastoma and 8 cholestasis

CD8+ T lymphocyte response against extrahepatic biliary epithelium is activated by epitopes within NSP4 in experimental biliary atresia.

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Identifikohuni Regjistrohu

Interferon (IFN)-γ-driven and CD8+ T cell-dependent inflammatory injury to extrahepatic biliary epithelium (EHBE) is likely to be involved in the development of biliary atresia (BA). We previously showed that viral protein NSP4 is the pathogenic immunogen that causes biliary injury in BA. In this

Antifibrotic potential of bone marrow‑derived mesenchymal stem cells in biliary atresia mice.

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Identifikohuni Regjistrohu

Biliary atresia (BA) is a rare and severe disease that affects infants where a fibroinflammatory process destroys the bile ducts, leading to fibrosis and biliary cirrhosis, and mortality if untreated. Bone marrow‑derived mesenchymal stem cells (BMMSCs) have been considered as a promising therapy in

Glutathione S-transferases in neonatal liver disease.

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Identifikohuni Regjistrohu

OBJECTIVE To investigate the distribution of alpha and pi class glutathione S-transferases (GST) in normal fetal, neonatal, and adult liver; and to examine changes in GST expression in neonatal liver disease. METHODS alpha and pi class GST were immunolocalised in sections of formalin fixed liver

Impaired redox and protein homeostasis as risk factors and therapeutic targets in toxin-induced biliary atresia

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Identifikohuni Regjistrohu

Background and aims: Extra-hepatic biliary atresia (BA) is a pediatric liver disease with no approved medical therapy. Recent studies using human samples and experimental modeling suggest that glutathione redox metabolism and heterogeneity play a role in disease

A phase 2 trial of N-Acetylcysteine in Biliary atresia after Kasai portoenterostomy.

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Identifikohuni Regjistrohu

Biliary atresia (BA) is a life-threatening liver disease of infancy, characterized by extrahepatic biliary obstruction, bile retention, and progressive liver injury. The Kasai portoenterostomy (KP) is BA's only nontransplant treatment. Its success is variable and depends on restoration

The synthetic toxin biliatresone causes biliary atresia in mice

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Identifikohuni Regjistrohu

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.

The Characteristics of Antioxidant Activity after Liver Transplantation in Biliary Atresia Patients.

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Identifikohuni Regjistrohu

OBJECTIVE Cholestatic liver injury is associated with a high production of free radicals. The pathogenesis of liver injury in biliary atresia (BA) patients is largely undefined. The goal of the present study was to clarify the oxidative damage and the changes in antioxidant enzyme activities that

The toxin biliatresone causes mouse extrahepatic cholangiocyte damage and fibrosis through decreased glutathione and SOX17.

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Identifikohuni Regjistrohu

Biliary atresia, the most common indication for pediatric liver transplantation, is a fibrotic disease of unknown etiology affecting the extrahepatic bile ducts of newborns. The recently described toxin biliatresone causes lumen obstruction in mouse cholangiocyte spheroids and represents a new model

Gene Expression Signatures Associated With Survival Times of Pediatric Patients With Biliary Atresia Identify Potential Therapeutic Agents.

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Identifikohuni Regjistrohu

Little is known about the factors that affect outcomes of patients with biliary atresia and there are no medical therapies that increase biliary drainage.Liver biopsies and clinical data were obtained from infants with cholestasis and from children without

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