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Related Experiment Video

Updated: Jul 10, 2026

Using Human Induced Pluripotent Stem Cell-derived Hepatocyte-like Cells for Drug Discovery
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Published on: May 19, 2018

Innovative models to explore hepatic involvement in Prader-Willi syndrome.

Romar Guintu Dabban1,2,3, Graziano Grugni4, Adele Bondesan4

  • 1Fondazione Italiana Fegato (Italian Liver Foundation), Innovative Models Unit, Trieste, Italy.

Frontiers in Endocrinology
|July 9, 2026
PubMed
Summary

Prader-Willi syndrome (PWS) involves unique metabolic changes, with impaired lipid handling affecting liver health despite obesity. Induced pluripotent stem cell models offer insights into PWS mechanisms and potential therapies.

Keywords:
Prader-Willi syndromehepatocyte-like cellsiPSCslipid metabolismliver organoids

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Human Liver Microphysiological System for Assessing Drug-Induced Liver Toxicity In Vitro

Published on: January 31, 2022

Area of Science:

  • Genetics
  • Metabolic Disorders
  • Hepatology

Background:

  • Prader-Willi syndrome (PWS) is a rare neurodevelopmental disorder with complex metabolic features, including obesity and altered lipid metabolism.
  • PWS exhibits an unusual metabolic profile with extreme adiposity but a lower risk of insulin resistance and hepatic complications.
  • Genetic factors in PWS impair adipocyte lipid handling, impacting hepatic lipid burden and potentially influencing steatosis risk.

Purpose of the Study:

  • To investigate the distinct metabolic signature and hepatic involvement in Prader-Willi syndrome.
  • To explore the role of intrinsic lipid handling and endocrine signaling in PWS-related liver issues.
  • To highlight the utility of induced pluripotent stem cell (iPSC)-derived models for studying PWS.

Main Methods:

  • Systemic lipidomic and metabolomic profiling to identify metabolic signatures.
  • Analysis of hepatic lipid handling and endocrine signaling pathways in PWS.
  • Utilizing hepatocyte-like cells (HLCs) and iPSC-derived organoids as models for PWS research.

Main Results:

  • PWS displays a unique metabolic signature with altered circulating phospholipids.
  • Hepatic involvement in PWS is primarily driven by intrinsic lipid handling defects, not solely adiposity.
  • Metabolic dysfunction-associated steatotic liver disease (MASLD) is less common in PWS.

Conclusions:

  • iPSC-derived models (HLCs, organoids) are crucial for dissecting PWS mechanisms, bridging molecular and organismal levels.
  • These models facilitate understanding patient-specific epigenetic regulation and tissue-specific metabolic dysfunction in PWS.
  • Advanced mechanistic understanding and therapeutic development for PWS can be achieved using these innovative stem cell-based strategies.