Xiong Qi, Ge Wei
National Key Laboratory of Medical Molecular Biology and Department of Immunology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, Beijing 100005, P.R. China; Department of Orthopedics, General Hospital of Chinese PLA, Beijing 100853, P.R. China.
National Key Laboratory of Medical Molecular Biology and Department of Immunology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, Beijing 100005, P.R. China.
Biomed Rep. 2016 Sep;5(3):277-282. doi: 10.3892/br.2016.729. Epub 2016 Jul 29.
Cushing's disease (CD) is a severe (and potentially fatal) disease caused by adrenocorticotropic hormone (ACTH)-secreting adenomas of the pituitary gland (often termed pituitary adenomas). The majority of ACTH-secreting corticotroph tumors are sporadic and CD rarely appears as a familial disorder, thus, the genetic mechanisms underlying CD are poorly understood. Studies have reported that various mutated genes are associated with CD, such as those in menin 1, aryl hydrocarbon receptor-interacting protein and the nuclear receptor subfamily 3 group C member 1. Recently it was identified that ubiquitin-specific protease 8 mutations contribute to CD, which was significant towards elucidating the genetic mechanisms of CD. The present study reviews the associated gene mutations in CD patients.
库欣病(CD)是一种由垂体分泌促肾上腺皮质激素(ACTH)的腺瘤(通常称为垂体腺瘤)引起的严重(且可能致命)疾病。大多数分泌ACTH的促肾上腺皮质激素肿瘤是散发性的,CD很少表现为家族性疾病,因此,对CD潜在的遗传机制了解甚少。研究报告称,多种突变基因与CD相关,如Menin 1、芳烃受体相互作用蛋白和核受体亚家族3 C组成员1中的基因。最近发现泛素特异性蛋白酶8突变与CD有关,这对阐明CD的遗传机制具有重要意义。本研究综述了CD患者的相关基因突变。