title: AIP and its interacting partners.
abstract: Germline mutations in the aryl hydrocarbon receptor-interacting protein gene (AIP) predispose to young-onset pituitary tumours, most often to GH- or prolactin-secreting adenomas, and most of these patients belong to familial isolated pituitary adenoma families. The molecular pathway initiated by the loss-of-function AIP mutations leading to pituitary tumour formation is unknown. AIP, a co-chaperone of heat-shock protein 90 and various nuclear receptors, belongs to the family of tetratricopeptide repeat (TPR)-containing proteins. It has three antiparallel α-helix motifs (TPR domains) that mediate the interaction of AIP with most of its partners. In this review, we summarise the known interactions of AIP described so far. The identification of AIP partners and the understanding of how AIP interacts with these proteins might help to explain the specific phenotype of the families with heterozygous AIP mutations, to gain deeper insight into the pathological process of pituitary tumour formation and to identify novel drug targets.
authors: Department of Endocrinology, Bart's and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK.
authors: Giampaolo
authors: G
authors: Trivellin
authors: Márta
authors: M
authors: Korbonits
mesh_terms: D000236:Adenoma / Q000235:genetics / Q000378:metabolism; D019309:Epstein-Barr Virus Nuclear Antigens / Q000235:genetics / Q000378:metabolism; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins / Q000235:genetics / Q000378:metabolism*; D010911:Pituitary Neoplasms / Q000235:genetics / Q000378:metabolism; D015534:Trans-Activators / Q000235:genetics / Q000378:metabolism; D054334:Viral Regulatory and Accessory Proteins
chemical_list: C508151:EBNA-3A antigen; D019309:Epstein-Barr Virus Nuclear Antigens; D047908:Intracellular Signaling Peptides and Proteins; D015534:Trans-Activators; D054334:Viral Regulatory and Accessory Proteins; C103265:aryl hydrocarbon receptor-interacting protein; C057628:hepatitis B virus X protein