Ibáñez-Costa Alejandro, Korbonits Márta
Centre for EndocrinologyWilliam Harvey Research Institute, Barts and The London School of Medicine, Queen Mary University of London, London, UK.
Centre for EndocrinologyWilliam Harvey Research Institute, Barts and The London School of Medicine, Queen Mary University of London, London, UK
J Endocrinol. 2017 Dec;235(3):R101-R116. doi: 10.1530/JOE-17-0254. Epub 2017 Aug 23.
Classic somatostatin analogues aimed at somatostatin receptor type 2, such as octreotide and lanreotide, represent the mainstay of medical treatment for acromegaly. These agents have the potential to decrease hormone secretion and reduce tumour size. Patients with a germline mutation in the aryl hydrocarbon receptor-interacting protein gene, , develop young-onset acromegaly, poorly responsive to pharmacological therapy. In this review, we summarise the most recent studies on AIP-related pituitary adenomas, paying special attention to the causes of somatostatin resistance; the somatostatin receptor profile including type 2, type 5 and truncated variants; the role of G proteins in this pathology; the use of first and second generation somatostatin analogues; and the role of ZAC1, a zinc-finger protein with expression linked to AIP in somatotrophinoma models and acting as a key mediator of octreotide response.
针对2型生长抑素受体的经典生长抑素类似物，如奥曲肽和兰瑞肽，是肢端肥大症药物治疗的主要手段。这些药物有可能减少激素分泌并缩小肿瘤大小。芳烃受体相互作用蛋白基因发生种系突变的患者会患早发性肢端肥大症，对药物治疗反应不佳。在这篇综述中，我们总结了关于AIP相关垂体腺瘤的最新研究，特别关注生长抑素抵抗的原因；生长抑素受体谱，包括2型、5型和截短变体；G蛋白在这种病理中的作用；第一代和第二代生长抑素类似物的使用；以及ZAC1的作用，ZAC1是一种锌指蛋白，其表达与生长激素瘤模型中的AIP相关，并作为奥曲肽反应的关键介质。
