title: GATA factors in endocrine neoplasia.
abstract: GATA transcription factors are structurally-related zinc finger proteins that recognize the consensus DNA sequence WGATAA (the GATA motif), an essential cis-acting element in the promoters and enhancers of many genes. These transcription factors regulate cell fate specification and differentiation in a wide array of tissues. As demonstrated by genetic analyses of mice and humans, GATA factors play pivotal roles in the development, homeostasis, and function of several endocrine organs including the adrenal cortex, ovary, pancreas, parathyroid, pituitary, and testis. Additionally, GATA factors have been shown to be mutated, overexpressed, or underexpressed in a variety of endocrine tumors (e.g., adrenocortical neoplasms, parathyroid tumors, pituitary adenomas, and sex cord stromal tumors). Emerging evidence suggests that GATA factors play a direct role in the initiation, proliferation, or propagation of certain endocrine tumors via modulation of key developmental signaling pathways implicated in oncogenesis, such as the WNT/β-catenin and TGFβ pathways. Altered expression or function of GATA factors can also affect the metabolism, ploidy, and invasiveness of tumor cells. This article provides an overview of the role of GATA factors in endocrine neoplasms. Relevant animal models are highlighted.
authors.affiliation: Children's Hospital, Helsinki University Central Hospital, University of Helsinki, 00290 Helsinki, Finland.
authors.forename: Marjut
authors.initials: M
authors.lastname: Pihlajoki
authors.affiliation: Children's Hospital, Helsinki University Central Hospital, University of Helsinki, 00290 Helsinki, Finland; Department of Obstetrics and Gynecology, Helsinki University Central Hospital, University of Helsinki, 00290 Helsinki, Finland.
authors.forename: Anniina
authors.initials: A
authors.lastname: Färkkilä
authors.affiliation: Children's Hospital, Helsinki University Central Hospital, University of Helsinki, 00290 Helsinki, Finland.
authors.forename: Tea
authors.initials: T
authors.lastname: Soini
authors.affiliation: Children's Hospital, Helsinki University Central Hospital, University of Helsinki, 00290 Helsinki, Finland; Department of Pediatrics, St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, MO 63110, USA.
authors.forename: Markku
authors.initials: M
authors.lastname: Heikinheimo
authors.affiliation: Department of Pediatrics, St. Louis Children's Hospital, Washington University School of Medicine, St. Louis, MO 63110, USA; Department of Developmental Biology, Washington University School of Medicine, St. Louis, MO 63110, USA. Electronic address: wilson_d@wustl.edu.
authors.forename: David B
authors.initials: DB
authors.lastname: Wilson
mesh_terms: D000818:Animals; D014408:Biomarkers, Tumor / Q000235:genetics / Q000378:metabolism; D004701:Endocrine Gland Neoplasms / Q000235:genetics* / Q000378:metabolism*; D050980:GATA Transcription Factors / Q000235:genetics* / Q000378:metabolism*; D006801:Humans; D051379:Mice; D009154:Mutation; D009361:Neoplasm Invasiveness; D009374:Neoplasms, Experimental; D011003:Ploidies; D016212:Transforming Growth Factor beta / Q000378:metabolism; D060449:Wnt Signaling Pathway
chemical_list: D014408:Biomarkers, Tumor; D050980:GATA Transcription Factors; D016212:Transforming Growth Factor beta
keywords: Adrenocortical tumor; Granulosa cell tumor; Parathyroid tumor; Pituitary adenoma; Sertoli–Leydig cell tumor