GABAergic regulation of Locus coeruleus activity in necdin-deficient mice, an animal model of Prader-Willi syndrome
Necdin deficiency selectively disrupts GABA<sub>B</sub> receptor-mediated tonic inhibition of LC-NE neurons while preserving GABA<sub>A</sub> receptor function. Elevated astrocytic proliferation and GABA release may further enhance ambient inhibition, contributing to LC hypoactivity and the neurobehavioral phenotypes of PWS. These findings identify GABA<sub>B</sub> receptor dysfunction and astrocytic dysregulation as potential mechanistic targets for therapeutic intervention in PWS.
Authors
- Shi-Bing Wong
- Hao Chan (ORCID: https://orcid.org/0000-0001-9771-1869)
- Sin-Jhong Cheng
- Li-Ping Tsai
- Ming-Yuan Min
- Chun-Hsien Yu
- Wei-Chen Hung
- Chen-En Yang
Institutions
- National Taiwan University (TW)
- Tzu Chi University (TW)
- Institute of Biomedical Sciences, Academia Sinica (TW)
- Taipei Tzu Chi Hospital (TW)
- Institute of Physics, Academia Sinica (TW)
- Taipei City Hospital (TW)
- Academia Sinica (TW)
Publication Details
- Journal
- Journal of Neurodevelopmental Disorders
- Published
- 2025-12-30
- DOI
- https://doi.org/10.1186/s11689-025-09667-9
- Primary Topic
- Genetic Syndromes and Imprinting
- Type
- article
- Field-Weighted Citation Impact
- 0.00