title: Minireview: transcriptional regulation of adrenocortical development.
abstract: The adrenal glands are comprised of two distinct endocrine organs: the outer cortex, which is derived from mesoderm and synthesizes steroid hormones, and the inner medulla, which contains neuroectodermal cells derived from the neural crest and produces the catecholamine hormones norepinephrine and epinephrine. The developmental program that gives rise to the adrenal gland begins early during embryogenesis and continues throughout gestation and well after birth. In this article, we review the molecular mechanisms of adrenal differentiation and development, focusing on the contributions of genes responsible for the development of the adrenal cortex as identified from studies of experimental animal models and human subjects with clinical diseases. These studies identify a hierarchical network of transcription factors, including Wilms' tumor-1, steroidogenic factor-1, dosage-sensitive sex reversal, adrenal hypoplasia congenita, X-linked-1, PBX1, and CITED2, that both give rise to the adrenal cortex and subsequently determine its subsequent function in steroidogenesis.
authors.affiliation: Division of Metabolism, Endocrinology, and Metabolism, Department of Internal Medicine, University of Michigan, 1150 West Medical Center Drive, Ann Arbor, Michigan 48109-0678, USA. ghammer@med.umich.edu
authors.forename: Gary D
authors.initials: GD
authors.lastname: Hammer
authors.forename: Keith L
authors.initials: KL
authors.lastname: Parker
authors.forename: Bernard P
authors.initials: BP
authors.lastname: Schimmer
mesh_terms: D000302:Adrenal Cortex / Q000196:embryology* / Q000502:physiology*; D000311:Adrenal Glands / Q000196:embryology* / Q000473:pathology; D000818:Animals; D057137:DAX-1 Orphan Nuclear Receptor; D004268:DNA-Binding Proteins / Q000502:physiology; D004837:Epinephrine / Q000378:metabolism; D018507:Gene Expression Regulation, Developmental; D018398:Homeodomain Proteins; D006801:Humans; D008648:Mesoderm / Q000378:metabolism; D051379:Mice; D008954:Models, Biological; D009638:Norepinephrine / Q000378:metabolism; D000075062:Pre-B-Cell Leukemia Transcription Factor 1; D011518:Proto-Oncogene Proteins / Q000502:physiology; D018160:Receptors, Cytoplasmic and Nuclear; D018168:Receptors, Retinoic Acid / Q000502:physiology; D012097:Repressor Proteins / Q000502:physiology; D054339:Steroidogenic Factor 1; D013256:Steroids / Q000378:metabolism; D015534:Trans-Activators / Q000502:physiology; D014157:Transcription Factors / Q000378:metabolism / Q000502:physiology; D014158:Transcription, Genetic; D025721:WT1 Proteins / Q000502:physiology
chemical_list: C486381:CITED2 protein, human; D057137:DAX-1 Orphan Nuclear Receptor; D004268:DNA-Binding Proteins; D018398:Homeodomain Proteins; C539965:NR0B1 protein, human; C515057:NR5A1 protein, human; C539966:Nr0b1 protein, mouse; D000075062:Pre-B-Cell Leukemia Transcription Factor 1; D011518:Proto-Oncogene Proteins; D018160:Receptors, Cytoplasmic and Nuclear; D018168:Receptors, Retinoic Acid; D012097:Repressor Proteins; D054339:Steroidogenic Factor 1; D013256:Steroids; D015534:Trans-Activators; D014157:Transcription Factors; D025721:WT1 Proteins; C083400:PBX1 protein, human; C515058:steroidogenic factor 1, mouse; D009638:Norepinephrine; D004837:Epinephrine