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Prader-Willi syndrome: A model for understanding the ghrelin system
Maithé Tauber1,2,3, Muriel Coupaye4, Gwenaelle Diene1,5
1Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, CHU Toulouse, Toulouse, France.
Journal of Neuroendocrinology
|May 3, 2019
Summary
Prader-Willi syndrome (PWS) is linked to high ghrelin levels, impacting appetite and obesity. Modulating the ghrelin system shows promise for treating PWS, a rare neurodevelopmental disorder.
Area of Science:
- Endocrinology
- Neuroscience
- Genetics
Background:
- Ghrelin, a gut peptide, regulates appetite, metabolism, and behavior.
- Prader-Willi syndrome (PWS) is characterized by severe obesity, hyperphagia, and elevated ghrelin levels.
- The role of hyperghrelinemia in PWS pathophysiology was initially unclear but is now better understood.
Purpose of the Study:
- To provide an overview of Prader-Willi syndrome.
- To detail the natural history of PWS, including recent ghrelin system data.
- To discuss therapeutic strategies targeting the ghrelin system in PWS.
Main Methods:
- Review of current literature on PWS and the ghrelin system.
- Analysis of natural history data in PWS patients.
- Discussion of therapeutic interventions modulating ghrelin signaling.
Main Results:
- PWS involves complex interactions between the ghrelin system and neurodevelopmental pathways.
- Hyperghrelinemia contributes to the characteristic hyperphagia and adiposity in PWS.
- Modulating the ghrelin system presents a promising therapeutic avenue for PWS.
Conclusions:
- The ghrelin system plays a significant role in the pathophysiology of Prader-Willi syndrome.
- Understanding the ghrelin system's dysfunction is crucial for developing effective PWS treatments.
- Targeting ghrelin signaling offers potential for managing PWS symptoms, including obesity and hyperphagia.
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