Concept

Progressive familial intrahepatic cholestasis

Related publications (15)

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

Dissecting the role of biliary epithelial cells during non-alcoholic fatty liver disease progression

Ece Yildiz

The liver is the largest solid organ and the only one capable of using regenerative mechanisms to recover its mass fully. Although liver regeneration from acute injuries has been effective and extensively studied, chronic liver damage has adverse effects o ...

EPFL2022

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 PUBLISHING GROUP2020

In vivo detection of gamma-glutamyl-transferase up-regulation in glioma using hyperpolarized gamma-glutamyl-[1-C-13]glycine

Hikari Ananda Infinity Yoshihara

Glutathione (GSH) is often upregulated in cancer, where it serves to mitigate oxidative stress. gamma-glutamyl-transferase (GGT) is a key enzyme in GSH homeostasis, and compared to normal brain its expression is elevated in tumors, including in primary gli ...

NATURE PUBLISHING GROUP2020

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

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

Raised hepatic bile acid concentrations during pregnancy in mice are associated with reduced farnesoid X receptor function

Kristina Schoonjans

Pregnancy alters bile acid homeostasis and can unmask cholestatic disease in genetically predisposed but otherwise asymptomatic individuals. In this report, we show that normal pregnant mice have raised hepatic bile acid levels in the presence of procholes ...

2010

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