Concept

Alagille syndrome

Related publications (12)

Structural basis of bile salt extrusion and small-molecule inhibition in human BSEP

Henning Paul-Julius Stahlberg, Dongchun Ni

BSEP (ABCB11) is an ATP-binding cassette transporter that is expressed in hepatocytes and extrudes bile salts into the canaliculi of the liver. BSEP dysfunction, caused by mutations or induced by drugs, is frequently associated with severe cholestatic live ...

Berlin2023

Engineering Modular 3D Liver Culture Microenvironments In Vitro to Parse the Interplay between Biophysical and Biochemical Microenvironment Cues on Hepatic Phenotypes

Ece Yildiz

In vitro models of human liver functions are used across a diverse range of applications in preclinical drug development and disease modeling, with particular increasing interest in models that capture facets of liver inflammatory status. This study invest ...

WILEY-V C H VERLAG GMBH2022

The G Protein-Coupled Bile Acid Receptor TGR5 (Gpbar1) Modulates Endothelin-1 Signaling in Liver

Kristina Schoonjans

TGR5 (Gpbar1) is a G protein-coupled receptor responsive to bile acids (BAs), which is expressed in different non-parenchymal cells of the liver, including biliary epithelial cells, liver-resident macrophages, sinusoidal endothelial cells (LSECs), and acti ...

2019

Probing perturbed hepatic metabolism in bile-duct-ligated rats with hyperpolarized 13C pyruvate and arginine

Rolf Gruetter, Cristina Ramona Cudalbu, Dmitri Firsov, Hikari Ananda Infinity Yoshihara

Detoxification of ammonia by the urea cycle and maintenance of glucose homeostasis by gluconeogenesis are two critical functions of the liver. The bile duct ligation (BDL) model of cirrhosis was used to test the ability of hyperpolarized [6-13C]arginine an ...

2018

Microencapsulated human mesenchymal stem cells decrease liver fibrosis in mice

Jutta Christine Wandrey, Redouan Mahou, Yannick Müller

Background & Aims: Mesenchymal stem cell (MSC) transplantation was shown to be effective for the treatment of liver fibrosis, but the mechanisms of action are not yet fully understood. We transplanted encapsulated human MSCs in two mouse models of liver fi ...

Elsevier Science Bv2015

Phosphorylation of the nuclear receptor co-repressor 1 by protein kinase B (PKB/Akt) switches its co-repressor targets in the liver

Johan Auwerx, Kristina Schoonjans, Dongryeol Ryu, Xu Wang, Adriano Maida

The nuclear receptor corepressor 1 (NCoR1) is a transcriptional co-regulator that has wide-ranging effects on gene expression patterns. In the liver, NCoR1 represses lipid synthesis in the fasting state, whereas it inhibits the activation of PPARα upon fee ...

Wiley-Blackwell2015

The Receptor TGR5 Protects the Liver From Bile Acid Overload During Liver Regeneration in Mice

Kristina Schoonjans

Many regulatory pathways are involved in liver regeneration after partial hepatectomy (PH) to initiate growth, protect liver cells, and sustain functions of the remnant liver. Bile acids (BAs), whose levels rise in the blood early after PH, stimulate both ...

Wiley-Blackwell2013

Jagged1 in the portal vein mesenchyme regulates intrahepatic bile duct development: insights into Alagille syndrome

Freddy Radtke

Mutations in the human Notch ligand jagged 1 (JAG1) result in a multi-system disorder called Alagille syndrome (AGS). AGS is chiefly characterized by a paucity of intrahepatic bile ducts (IHBD), but also includes cardiac, ocular, skeletal, craniofacial and ...

2010

Liver-specific inactivation of Notch2, but not Notch1, compromises intrahepatic bile duct development in mice

Freddy Radtke

The Notch pathway is an evolutionary conserved, intercellular signaling pathway that plays an important role in cell fate specification and the embryonic development of many organs, including the liver. In humans, mutations in the Notch receptor ligand Jag ...

2008

Inducible inactivation of Notch1 causes nodular regenerative hyperplasia in mice

Freddy Radtke, Xiao Wang

The discovery that the human Jagged1 gene (JAG1) is the Alagille syndrome disease gene indicated that Notch signaling has an important role in bile duct homeostasis. The functional study of this signaling pathway has been difficult because mice with target ...

2005

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