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首页> 外文期刊>Journal of neuroendocrinology >Prader‐Willi syndrome: A model for understanding the ghrelin system
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Prader‐Willi syndrome: A model for understanding the ghrelin system

机译：PRADER-WILLI综合征：理解GHRELIN系统的模型

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Abstract Subsequent to the discovery of ghrelin as the endogenous ligand of growth hormone secretagogue receptor 1a, this unique gut peptide has been found to exert numerous physiological effects, such as appetite stimulation and lipid accumulation via the central regulating mechanisms in the hypothalamus, stimulation of gastric motility, regulation of glucose metabolism and brown fat thermogenesis, and modulation of stress, anxiety, taste sensation, reward‐seeking behaviour and the sleep/wake cycle. Prader‐Willi syndrome (PWS) has been described as a unique pathological state characterised by severe obesity and high circulating levels of ghrelin. It was hypothesised that hyperghrelinaemia would explain at least a part of the feeding behaviour and body composition of PWS patients, who are characterised by hyperphagia, an obsession with food and food‐seeking, and increased adiposity. Initially, the link between hyperghrelinaemia and growth hormone deficiency, which is observed in 90% of the children with PWS, was not fully understood. Over the years, however, the increasing knowledge on ghrelin, PWS features and the natural history of the disease has led to a more comprehensive description of the abnormal ghrelin system and its role in the pathophysiology of this rare and complex neurodevelopmental genetic disease. In the present study, we (a) present the current view of PWS; (b) explain its natural history, including recent data on the ghrelin system in PWS patients; and (c) discuss the therapeutic approach of modulating the ghrelin system in these patients and the first promising results.
机译：摘要在将Ghrelin发现作为生长激素的内源性配体，随后被发现，这种独特的肠肽已被发现施加许多生理效应，例如通过下丘脑中的中央调节机制，胃的中央调节机制促进食欲刺激和脂质积累。葡萄糖代谢和棕色脂肪热量的动力，调节，压力，焦虑，味觉，奖励行为和睡眠/唤醒周期的调节。 PRADER-WILLI综合征（PWS）被描述为一种独特的病理状态，其特征是肥胖的严重肥胖和高循环水平的GHRELIN。假设高血脂血症将至少解释饲养行为和PWS患者的身体组成的一部分，其特征是患者，患有胃肠，对食物和食品的痴迷以及增加的肥胖。最初，HyperGhrelina血症和生长激素缺乏之间的联系，在90％的患有PWS的儿童中观察到的，并未完全理解。然而，多年来，对Ghrelin，PWS特征和疾病的自然历史的越来越多的知识导致了更全面的Ghrelin系统描述及其在这种稀有和复杂的神经发育遗传疾病的病理生理学中的作用。在本研究中，我们（a）介绍了PWS的当前观点; （b）解释其自然历史，包括近期PWS患者Ghrelin系统的数据; （c）讨论在这些患者中调节Ghrelin系统的治疗方法和第一个有前途的结果。

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