====== Aryl hydrocarbon interacting protein ====== The [[AIP gene]] provides instructions for making a protein called [[aryl hydrocarbon interacting protein]] [[Familial isolated pituitary neuroendocrine tumor]] (FIPA) is an autosomal dominant disease, characterized by low penetrance, early-onset disease, more invasive tumor growth, as well as somatotroph and lactotroph adenomas in most cases. It has been indicated that the aryl hydrocarbon receptor interacting protein (AIP) gene is a tumor suppressor gene. The exact molecular mechanism by which its disfunction promotes tumorigenesis of pituitary is unclear ((Cai F, Zhang YD, Dai CX, Liu XH, Yang YK, Yao Y, Wang RZ. [Aryl hydrocarbon receptor interacting protein gene and familial isolated pituitary neuroendocrine tumors]. Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2012 Dec;34(6):640-4. doi: 10.3881/j.issn.1000-503X.2012.06.021. Review. Chinese. PubMed PMID: 23286415. )). Germline aryl hydrocarbon receptor-interacting protein (AIP) mutations are present in 15-30% of familial isolated pituitary neuroendocrine tumor (FIPA) families, and are responsible for 30% of pituitary [[gigantism]] cases (1). However, pathological accelerated growth and/or tall stature can be unrelated to the [[growth hormone]] (GH) axis, and may occur in isolation or as part of a syndrome, such as in Klinefelter, Marfan or Sotos syndromes (2). Marques et al. report a five-generation kindred with two brothers with pituitary gigantism due to AIP mutation-positive GH-secreting pituitary neuroendocrine tumors and their first-cousin coincidently also having gigantism due to Marfan syndrome ((Marques P, Collier D, Barkan A, Korbonits M. Coexisting pituitary and non-pituitary gigantism in the same family. Clin Endocrinol (Oxf). 2018 Sep 17. doi: 10.1111/cen.13852. [Epub ahead of print] PubMed PMID: 30223298. )).