Butler Merlin G, Bittel Douglas C
Section of Medical Genetics and Molecular Medicine, Children's Mercy Hospitals and Clinics and University of Missouri-Kansas City School of Medicine, Kansas City, Missouri 64108, USA.
Am J Med Genet A. 2007 Mar 1;143A(5):415-21. doi: 10.1002/ajmg.a.31687.
Prader-Willi syndrome (PWS) is an obesity syndrome characterized by rapid weight gain and excessive food intake. Food intake is regulated by the hypothalamus but directly influenced by gastrointestinal peptides responding to the nutritional status and body composition of an individual. Ghrelin, derived from preproghrelin, is secreted by the stomach and increases appetite while obestatin, a recently identified peptide derived post-translationally from preproghrelin, works in opposition to ghrelin by decreasing appetite. The objective of this study was to measure fasting obestatin and ghrelin levels in peripheral blood of subjects with PWS and compare to age and gender matched control subjects. Plasma obestatin and ghrelin levels were measured in subjects with PWS (n = 16, mean age = 16.0 +/- 13.3 years; age range 1-44 years) and age and gender matched control subjects (n = 16). Significantly higher obestatin levels were seen in the 16 PWS subjects (398 +/- 102 pg/ml) compared with 16 controls (325 +/- 109 pg/ml; matched t-test, P = 0.04), particularly in 5 young (< or =3 years old) PWS subjects (460 +/- 49 pg/ml) compared with 5 young controls (369 +/- 96 pg/ml; matched t-test, P = 0.03). No significant difference in ghrelin levels was seen between the PWS and comparison groups. No significant correlation was observed for either peptide when compared with body mass index but a significant negative correlation was seen for ghrelin and age in PWS subjects. Our observations suggest that obestatin may be higher in infants with PWS compared to comparison infants. The possibility that obestatin may contribute to the failure to thrive which is common in infants with PWS warrants further investigation.
普拉德-威利综合征（PWS）是一种肥胖综合征，其特征为体重快速增加和食物摄入量过多。食物摄入由下丘脑调节，但直接受胃肠道肽的影响，这些肽对个体的营养状况和身体组成做出反应。胃饥饿素由前胃饥饿素原衍生而来，由胃分泌，可增加食欲，而肥胖抑制素是最近发现的一种从前胃饥饿素原翻译后衍生的肽，通过降低食欲与胃饥饿素起相反作用。本研究的目的是测量PWS患者外周血中的空腹肥胖抑制素和胃饥饿素水平，并与年龄和性别匹配的对照受试者进行比较。对PWS患者（n = 16，平均年龄 = 16.0 +/- 13.3岁；年龄范围1 - 44岁）和年龄及性别匹配的对照受试者（n = 16）测量了血浆肥胖抑制素和胃饥饿素水平。16名PWS患者的肥胖抑制素水平（398 +/- 102 pg/ml）显著高于16名对照者（325 +/- 109 pg/ml；配对t检验，P = 0.04），特别是5名年轻（≤3岁）PWS患者（460 +/- 49 pg/ml）高于5名年轻对照者（369 +/- 96 pg/ml；配对t检验，P = 0.03）。PWS组和对照组之间的胃饥饿素水平未见显著差异。与体重指数相比，两种肽均未观察到显著相关性，但PWS患者中胃饥饿素与年龄呈显著负相关。我们的观察结果表明，与对照婴儿相比，PWS婴儿的肥胖抑制素水平可能更高。肥胖抑制素可能导致PWS婴儿常见的生长发育不良这一可能性值得进一步研究。
