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.
Authors
- Pim J. Koelink (ORCID: https://orcid.org/0000-0001-7048-9014)
- Sas Dijk
- Ronald P.J. Oude Elferink (ORCID: https://orcid.org/0000-0002-1779-0833)
- Norman Junge (ORCID: https://orcid.org/0000-0002-3099-2667)
- Georg F. Vogel (ORCID: https://orcid.org/0000-0002-2515-4490)
- Joris I. Erdmann (ORCID: https://orcid.org/0000-0001-8233-1505)
- Stan F.J. van de Graaf (ORCID: https://orcid.org/0000-0003-4238-4359)
- Kam S. Ho‐Mok
- Shu‐Hao Hsu (ORCID: https://orcid.org/0000-0002-9444-4192)
- Huey‐Ling Chen (ORCID: https://orcid.org/0000-0002-4074-5838)
- Weng Chuan Peng (ORCID: https://orcid.org/0000-0001-5467-5458)
- Zhixiong Ying (ORCID: https://orcid.org/0000-0002-3540-7877)
- Coen C. Paulusma (ORCID: https://orcid.org/0000-0001-7284-1974)
- Suzanne Duijst
- Wietse In het Panhuis (ORCID: https://orcid.org/0000-0002-4394-6387)
- Jung‐Chin Chang (ORCID: https://orcid.org/0000-0001-9973-188X)
- Arthur J. Verhoeven
- Björn Hartleben
- Jolie F. van der Heiden
Institutions
- 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)
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
Funders
- Amsterdam University Medical Centers
- National Taiwan University