Structures of human bile acid exporter ABCB11 reveal a transport mechanism facilitated by two tandem substrate-binding pockets

L Wang, WT Hou, J Wang, D Xu, C Guo, L Sun, K Ruan… - Cell Research, 2022 - nature.com
Dear Editor, Bile acids are a group of cholesterol-derived physiological surfactants required
for digestion of lipids and fat-soluble vitamins. 1 However, excess of bile acids is detrimental …

Cryo-EM structure of human bile salts exporter ABCB11

L Wang, WT Hou, L Chen, YL Jiang, D Xu, L Sun… - Cell Research, 2020 - nature.com
Dear Editor, Bile is a complex aqueous secretion produced by the liver that facilitates the
digestion of lipids in the small intestine. A group of ATP binding cassette (ABC) transporters …

[PDF][PDF] Zebrafish abcb11b mutant reveals strategies to restore bile excretion impaired by bile salt export pump deficiency

JL Ellis, KE Bove, EG Schuetz, D Leino… - …, 2018 - Wiley Online Library
Bile salt export pump (BSEP) adenosine triphosphate–binding cassette B11 (ABCB11) is a
liver‐specific ABC transporter that mediates canalicular bile salt excretion from hepatocytes …

[HTML][HTML] In vitro rescue of the bile acid transport function of ABCB11 variants by CFTR potentiators

E Mareux, M Lapalus, A Ben Saad, R Zelli… - International Journal of …, 2022 - mdpi.com
ABCB11 is responsible for biliary bile acid secretion at the canalicular membrane of
hepatocytes. Variations in the ABCB11 gene cause a spectrum of rare liver diseases. The …

Genetic variations of the ABC transporter gene ABCB11 encoding the human bile salt export pump (BSEP) in a Japanese population

SR Kim, Y Saito, M Itoda, K Maekawa… - Drug metabolism and …, 2009 - jstage.jst.go.jp
The bile salt export pump (BSEP) encoded by ABCB11 is located in the canalicular
membrane of hepatocytes and mediates the secretion of numerous conjugated bile salts into …

Biosynthesis and trafficking of the bile salt export pump, BSEP: therapeutic implications of BSEP mutations

CJ Soroka, JL Boyer - Molecular aspects of medicine, 2014 - Elsevier
The bile salt export pump (BSEP, ABCB11) is the primary transporter of bile acids from the
hepatocyte to the biliary system. This rate-limiting step in bile formation is essential to the …

Impaired expression and function of the bile salt export pump due to three novel ABCB11 mutations in intrahepatic cholestasis

J Noe, GA Kullak-Ublick, W Jochum, B Stieger… - Journal of …, 2005 - Elsevier
BACKGROUND/AIMS: Inherited dysfunction of the bile salt export pump BSEP (ABCB11)
causes a progressive and a benign form of familial intrahepatic cholestasis, denominated as …

Hepatic overexpression of Abcb11 in mice promotes the conservation of bile acids within the enterohepatic circulation

AS Henkel, KER Gooijert, R Havinga… - American Journal …, 2013 - journals.physiology.org
The bile salt export pump, encoded by ABCB11, is the predominant canalicular transport
protein for biliary bile acid secretion. The level of ABCB11 expression in humans is widely …

Expression Analysis of ATP‐Binding Cassette Transporters ABCB11 and ABCB4 in Primary Sclerosing Cholangitis and Variety of Pediatric and Adult Cholestatic and …

C Thoeni, R Waldherr, J Scheuerer… - Canadian Journal of …, 2019 - Wiley Online Library
ATP‐binding cassette (ABC) transporters are the members of the efflux pumps that are
responsible for the removal of cytotoxic substances by active transport. ABCB11, the bile salt …

A novel mutation within a transmembrane helix of the bile salt export pump (BSEP, ABCB11) with delayed development of cirrhosis

J Stindt, P Ellinger, K Weissenberger… - Liver …, 2013 - Wiley Online Library
Abstract Background & Aims The bile salt export pump (BSEP, ABCB 11) is essential for bile
salt secretion at the canalicular membrane of liver cells. Clinical phenotypes associated with …