Abstract
Prader-Willi syndrome (PWS), a congenital genetic disorder, has received attention due to its features, including hyperphagic behavior with a unique genetic background. While clinical research on this syndrome has focused on the management of the endocrinological and metabolic manifestations, there is growing evidence for the involvement of the brain in the pathogenesis of developmental and behavioral characteristics. Furthermore, researchers have investigated the genetic influence on molecular and cellular processes related to behavior using mouse models. On the other hand, modern advanced magnetic resonance imaging, three-dimensional high-resolution structural imaging, diffusion imaging, and resting-state functional imaging have revealed structural and functional alterations in the brains of individuals with PWS, supported by updated optimization and statistical methods. This chapter discusses research on the brain in PWS and addresses its contribution to hyperphagia, which leads to obesity, as well as the neural mechanisms that may underlie the behavioral phenotypes of this syndrome.
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Abbreviations
- 5-HT:
-
5-hydroxytryptamine
- AT:
-
Adipose tissue
- AVP:
-
Arginine-vasopressin
- cDN:
-
Cerebellar dentate nucleus
- DTI:
-
Diffusion tensor imaging
- ERP:
-
Event-related potential
- GABA:
-
Gamma amino butyric acid
- IPWSO:
-
International Prader-Willi Syndrome Organization
- MRI:
-
Magnetic resonance imaging
- MRS:
-
Magnetic resonance spectroscopy
- OXT:
-
Oxytocin
- PET:
-
Positron emission tomography
- PWS:
-
Prader-Willi syndrome
- snoRNA:
-
Small nucleolar RNA
- SPECT:
-
Single-photon emission computed tomography
- TD:
-
typically developing
- UPD:
-
Uniparental disomy
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Yamada, K. (2022). The Brain in Prader-Willi Syndrome. In: Patel, V., Preedy, V. (eds) Eating Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-67929-3_75-1
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