Publications associées à Cholestase intrahépatique familiale progressive à activité gamma-glutamyl-transférase basse

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 ...

Nature Portfolio2023

Transcriptomic analysis across liver diseases reveals disease-modulating activation of constitutive androstane receptor in cholestasis

Kristina Schoonjans, Jian Liu, Bhoomika Mathur

Background & Aims: Liver diseases are caused by many factors, such as genetics, nutrition, and viruses. Therefore, it is important to delineate transcriptomic changes that occur in various liver diseases. Methods: We performed high-throughput sequencing of ...

2020

Maternal glucose homeostasis is impaired in mouse models of gestational cholestasis

Kristina Schoonjans

Women with intrahepatic cholestasis of pregnancy (ICP), a disorder characterised by raised serum bile acids, are at increased risk of developing gestational diabetes mellitus and have impaired glucose tolerance whilst cholestatic. FXR and TGR5 are modulato ...

Nature Research2020

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

Direct Monitoring of γ-Glutamyl Transpeptidase Activity In Vivo Using a Hyperpolarized (13) C-Labeled Molecular Probe

Arnaud Comment, Josefina Adriana Maria Bastiaansen, Hikari Ananda Infinity Yoshihara, Yuhei Takado, Emine Can

The γ-glutamyl transpeptidase (GGT) enzyme plays a central role in glutathione homeostasis. Direct detection of GGT activity could provide critical information for the diagnosis of several pathologies. We propose a new molecular probe, γ-Glu-[1-(13) C]Gly, ...

Wiley-Blackwell2016

Intestinal FXR agonism promotes adipose tissue browning and reduces obesity and insulin resistance

Kristina Schoonjans, Alessia Perino

The systemic expression of the bile acid (BA) sensor farnesoid X receptor (FXR) has led to promising new therapies targeting cholesterol metabolism, triglyceride production, hepatic steatosis and biliary cholestasis. In contrast to systemic therapy, bile a ...

Nature Publishing Group2015

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