Endocrine Research Unit, Medizinische Klinik Campus Innenstadt, Klinikum der LMU, Ziemssenstr. 1, D-80336 Munich, Germany.
Mol Cell Endocrinol. 2012 Mar 31;351(1):78-86. doi: 10.1016/j.mce.2011.09.045. Epub 2011 Nov 11.
Over the past decade, research on human adrenocortical neoplasia has been dominated by gene expression profiling of tumor specimens and by analysis of genetic disorders associated with a predisposition to these tumors. Although these studies have identified key genes and associated signaling pathways that are dysregulated in adrenocortical neoplasms, the molecular events accounting for the frequent occurrence of benign tumors and low rate of malignant transformation remain unknown. Moreover, the prognosis for patients with adrenocortical carcinoma remains poor, so new medical treatments are needed. Naturally occurring and genetically engineered animal models afford a means to investigate adrenocortical tumorigenesis and to develop novel therapeutics. This comparative review highlights adrenocortical tumor models useful for either mechanistic studies or preclinical testing. Three model species - mouse, ferret, and dog - are reviewed, and their relevance to adrenocortical tumors in humans is discussed.
在过去的十年中，人类肾上腺皮质肿瘤的研究主要集中在肿瘤标本的基因表达谱分析和与这些肿瘤易感性相关的遗传疾病分析上。尽管这些研究已经确定了在肾上腺皮质肿瘤中失调的关键基因和相关信号通路，但导致良性肿瘤频繁发生和恶性转化率低的分子事件仍不清楚。此外，肾上腺皮质癌患者的预后仍然很差，因此需要新的治疗方法。天然存在和基因工程的动物模型为研究肾上腺皮质肿瘤的发生和开发新的治疗方法提供了一种手段。本综述重点介绍了用于机制研究或临床前测试的肾上腺皮质肿瘤模型。综述了三种模型物种——鼠、雪貂和犬，并讨论了它们与人类肾上腺皮质肿瘤的相关性。
