Generation and characterization of a mouse model for bile salt export pump deficiency with the p.E297G mutation

BSEP<sup>E297G</sup> mice display a BSEP deficiency phenotype with a strongly reduced hepatobiliary bile acid transport capacity. The expression of immature BSEP protein suggests the potential to assess correctors of the BSEP functionality in vivo.

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Publication Details

Journal
Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids
Published
2025-10-18
DOI
https://doi.org/10.1016/j.bbalip.2025.159697
Primary Topic
Drug Transport and Resistance Mechanisms
Type
article
Field-Weighted Citation Impact
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Generation and characterization of a mouse model for bile salt export pump deficiency with the p.E297G mutation

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Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids
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Generation and characterization of a mouse model for bile salt export pump deficiency with the p.E297G mutation

Nicolette Huijkman, Martijn Koehorst, Bart van de Sluis, Henkjan J. Verkade, Folkert Kuipers, Antonia Felzen, Milaine V. Hovingh, Willemien F. J. Hof, H. de Vries, Niels Kloosterhuis, Jan Freark de Boer, Niels L. Mulder, Rick Havinga, Marieke Smit
article en

Abstract

BSEP<sup>E297G</sup> mice display a BSEP deficiency phenotype with a strongly reduced hepatobiliary bile acid transport capacity. The expression of immature BSEP protein suggests the potential to assess correctors of the BSEP functionality in vivo.

Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of LipidsVol. 1871(1)
University Medical Center Groningen (NL), University of Groningen (NL)
Openalex Percentile: Top 60%
Drug Transport and Resistance Mechanisms
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