Hypothalamic Neuropeptides and Regulation of Fat Mass in Prader-Willi Syndrome

2003 ◽  
pp. 31-43 ◽  
Author(s):  
A.P. Goldstone ◽  
U.A. Unmehopa ◽  
E.L. Thomas ◽  
A.E. Brynes ◽  
J.D. Bell ◽  
...  
Keyword(s):  
Fat Mass ◽  
Prader Willi Syndrome ◽  
Hypothalamic Neuropeptides

scholarly journals The Sun’s Vitamin in Adult Patients Affected by Prader–Willi Syndrome

Nutrients ◽  
2020 ◽  
Vol 12 (4) ◽  
pp. 1132
Author(s):  
Luigi Barrea ◽  
Giovanna Muscogiuri ◽  
Gabriella Pugliese ◽  
Sara Aprano ◽  
Giulia de Alteriis ◽  
...  
Keyword(s):  
Vitamin D ◽  
Waist Circumference ◽  
Fat Mass ◽  
Geographical Area ◽  
Dietary Vitamin ◽  
Prader Willi Syndrome ◽  
25Ohd Level ◽  
Vitamin D Intake ◽  
Increased Risk ◽  
Dietary Vitamin D

Prader–Willi syndrome (PWS) is a genetic disorder characterized by hyperphagia with progressive, severe obesity, and an increased risk of obesity-related comorbidities in adult life. Although low dietary vitamin D intake and low 25-hydroxy vitamin D (25OHD) levels are commonly reported in PWS in the context of bone metabolism, the association of low 25OHD levels with fat mass has not been extensively evaluated in PWS adults. The aims of this study were to investigate the following in PWS adults: (1) 25OHD levels and the dietary vitamin D intake; (2) associations among 25OHD levels with anthropometric measurements and fat mass; (3) specific cut-off values for body mass index (BMI) and fat mass predictive of the 25OHD levels. In this cross-sectional, single-center study we enrolled 30 participants, 15 PWS adults (age 19–41 years and 40% males) and 15 control subjects matched by age, sex, and BMI from the same geographical area (latitude 40° 49’ N; elevation 17 m). Fat mass was assessed using a bioelectrical impedance analysis (BIA) phase-sensitive system. The 25OHD levels were determined by a direct competitive chemiluminescence immunoassay. Dietary vitamin D intake data was collected by three-day food records. The 25OHD levels in the PWS adults were constantly lower across all categories of BMI and fat mass compared with their obese counterpart. The 25OHD levels were negatively associated with BMI (p = 0.04), waist circumference (p = 0.03), fat mass (p = 0.04), and dietary vitamin D intake (p < 0.001). During multiple regression analysis, dietary vitamin D intake was entered at the first step (p < 0.001), thus explaining 84% of 25OHD level variability. The threshold values of BMI and fat mass predicting the lowest decrease in the 25OHD levels were found at BMI ≥ 42 kg/m2 (p = 0.01) and fat mass ≥ 42 Kg (p = 0.003). In conclusion, our data indicate that: (i) 25OHD levels and dietary vitamin D intake were lower in PWS adults than in the control, independent of body fat differences; (ii) 25OHD levels were inversely associated with BMI, waist circumference, and fat mass, but low dietary vitamin D intake was the major determinant of low vitamin D status in these patients; (iii) sample-specific cut-off values of BMI and fat mass might help to predict risks of the lowest 25OHD level decreases in PWS adults. The presence of trained nutritionists in the integrated care teams of PWS adults is strongly suggested in order to provide an accurate nutritional assessment and tailored vitamin D supplementations.

scholarly journals Adult-onset deletion of the Prader-Willi syndrome susceptibility gene Snord116 in mice results in reduced feeding and increased fat mass

2017 ◽  
Vol 6 (2) ◽  
pp. 88-97 ◽  
Author(s):  
Louise Purtell ◽  
Yue Qi ◽  
Lesley Campbell ◽  
Amanda Sainsbury ◽  
Herbert Herzog
Keyword(s):  
Fat Mass ◽  
Susceptibility Gene ◽  
Prader Willi Syndrome ◽  
Adult Onset

scholarly journals Impaired Hypothalamic Regulation of Endocrine Function and Delayed Counterregulatory Response to Hypoglycemia in Magel2-Null Mice

Endocrinology ◽  
2011 ◽  
Vol 152 (3) ◽  
pp. 967-978 ◽  
Author(s):  
Alysa A. Tennese ◽  
Rachel Wevrick
Keyword(s):  
Fat Mass ◽  
Gh Deficiency ◽  
Genetic Disorder ◽  
Endocrine Function ◽  
Prader Willi Syndrome ◽  
Endocrine Dysfunction ◽  
Hypothalamic Dysfunction ◽  
Igf I ◽  
Hypothalamic Regulation ◽  
Corticosterone Response

Hypothalamic dysfunction may underlie endocrine abnormalities in Prader-Willi syndrome (PWS), a genetic disorder that features GH deficiency, obesity, and infertility. One of the genes typically inactivated in PWS, MAGEL2, is highly expressed in the hypothalamus. Mice deficient for Magel2 are obese with increased fat mass and decreased lean mass and have blunted circadian rhythm. Here, we demonstrate that Magel2-null mice have abnormalities of hypothalamic endocrine axes that recapitulate phenotypes in PWS. Magel2-null mice had elevated basal corticosterone levels, and although male Magel2-null mice had an intact corticosterone response to restraint and to insulin-induced hypoglycemia, female Magel2-null mice failed to respond to hypoglycemia with increased corticosterone. After insulin-induced hypoglycemia, Magel2-null mice of both sexes became more profoundly hypoglycemic, and female mice were slower to recover euglycemia, suggesting an impaired hypothalamic counterregulatory response. GH insufficiency can produce abnormal body composition, such as that seen in PWS and in Magel2-null mice. Male Magel2-null mice had Igf-I levels similar to control littermates. Female Magel2-null mice had low Igf-I levels and reduced GH release in response to stimulation with ghrelin. Female Magel2-null mice did respond to GHRH, suggesting that their GH deficiency has a hypothalamic rather than pituitary origin. Female Magel2-null mice also had higher serum adiponectin than expected, considering their increased fat mass, and thyroid (T4) levels were low. Together, these findings strongly suggest that loss of MAGEL2 contributes to endocrine dysfunction of hypothalamic origin in individuals with PWS.

P12 Norditropin increases lean muscle, reduces fat mass and normalizes height in children with Prader-Willi Syndrome

2010 ◽  
Vol 20 ◽  
pp. S43
Author(s):  
A.-M. Kappelgaard ◽  
S. Farholt ◽  
C. Lammer ◽  
A.H. Petersen ◽  
F. Schmidt ◽  
...  
Keyword(s):  
Fat Mass ◽  
Prader Willi Syndrome

scholarly journals Hypothalamic neuropeptides and neurocircuitries in Prader Willi syndrome

2021 ◽  
Author(s):  
Felipe Correa da‐Silva ◽  
Eric Fliers ◽  
Dick Swaab ◽  
Chun‐Xia Yi
Keyword(s):  
Prader Willi Syndrome ◽  
Hypothalamic Neuropeptides

Prader-willi syndrome: Genetics and behavior

1996 ◽  
Vol 71 (4) ◽  
pp. 187-212 ◽  
Author(s):  
Travis Thompson ◽  
Merlin Butler ◽  
William MacLean ◽  
Beth Joseph
Keyword(s):  
Prader Willi Syndrome ◽  
And Behavior
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