ATP8B1 Deficiency Causes Phosphodiesterase 4‐Mediated Glucagon Resistance and Impaired Gluconeogenesis in Mouse and Human Liver

ATP8B1 deficiency causes upregulation of PDE4D in the liver of Atp8b1<sup>G308V/G308V</sup> mice and PFIC1 patients. PDE4D-mediated glucagon resistance impairs gluconeogenesis and stimulates compensatory glycogenolysis in Atp8b1<sup>G308V/G308V</sup> mice and PFIC1 patients. Our study reveals novel metabolic insights of ATP8B1 deficiency in PFIC1 patients.

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Publication Details

Journal
Liver International
Published
2025-08-25
DOI
https://doi.org/10.1111/liv.70306
Citations
1
Primary Topic
Drug Transport and Resistance Mechanisms
Type
article
Field-Weighted Citation Impact
1.05

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article

ATP8B1 Deficiency Causes Phosphodiesterase 4‐Mediated Glucagon Resistance and Impaired Gluconeogenesis in Mouse and Human Liver

Pim J. Koelink, Sas Dijk, Ronald P.J. Oude Elferink, Norman Junge et al.
1 citations
Liver International
Drug Transport and Resistance Mechanisms
1.05
article

ATP8B1 Deficiency Causes Phosphodiesterase 4‐Mediated Glucagon Resistance and Impaired Gluconeogenesis in Mouse and Human Liver

Pim J. Koelink, Sas Dijk, Ronald P.J. Oude Elferink, Norman Junge, Georg F. Vogel, Joris I. Erdmann, Stan F.J. van de Graaf, Kam S. Ho‐Mok, Shu‐Hao Hsu, Huey‐Ling Chen, Weng Chuan Peng, Zhixiong Ying, Coen C. Paulusma, Suzanne Duijst, Wietse In het Panhuis, Jung‐Chin Chang, Arthur J. Verhoeven, Björn Hartleben, Jolie F. van der Heiden
article en
1 citations

Abstract

ATP8B1 deficiency causes upregulation of PDE4D in the liver of Atp8b1<sup>G308V/G308V</sup> mice and PFIC1 patients. PDE4D-mediated glucagon resistance impairs gluconeogenesis and stimulates compensatory glycogenolysis in Atp8b1<sup>G308V/G308V</sup> mice and PFIC1 patients. Our study reveals novel metabolic insights of ATP8B1 deficiency in PFIC1 patients.

Liver InternationalVol. 45(9)
Innsbruck Medical University (AT), National Taiwan University (TW), University of Applied Sciences Utrecht (NL), Universität Innsbruck (AT), Utrecht University (NL), Medizinische Hochschule Hannover (DE), Amsterdam Neuroscience (NL), Princess Máxima Center (NL), National Taiwan University Hospital (TW), Amsterdam University Medical Centers (NL), Amsterdam University of Applied Sciences (NL), University of Amsterdam (NL)
Amsterdam University Medical Centers, National Taiwan University
Openalex Percentile: Top 18%
Drug Transport and Resistance Mechanisms
1.05
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