Minireview: Transcriptional Regulation of Adrenocortical Development

• Published in: Endocrinology (Philadelphia) Vol. 146; no. 3; pp. 1018 - 1024
• Main Authors: Hammer, Gary D., Parker, Keith L., Schimmer, Bernard P.
• Format: Journal Article
• Language: English
• Published: Bethesda, MD Endocrine Society 01.03.2005; Oxford University Press
• Subjects: Adrenal cortex; Adrenal Cortex - embryology; Adrenal Cortex - physiology; Adrenal glands; Adrenal Glands - embryology; Adrenal Glands - pathology; Animal models; Animals; Biological and medical sciences; Catecholamine; Catecholamines; Cell differentiation; Chemical synthesis; DAX-1 Orphan Nuclear Receptor; DNA-Binding Proteins - physiology; Embryogenesis; Embryonic growth stage; Epinephrine - metabolism; Fundamental and applied biological sciences. Psychology; Gene Expression Regulation, Developmental; Gene regulation; Homeodomain Proteins; Hormones; Humans; Hypoplasia; Mesoderm; Mesoderm - metabolism; Mice; Models, Biological; Molecular modelling; Neural crest; Norepinephrine; Norepinephrine - metabolism; Pre-B-Cell Leukemia Transcription Factor 1; Proto-Oncogene Proteins - physiology; Receptors, Cytoplasmic and Nuclear; Receptors, Retinoic Acid - physiology; Repressor Proteins - physiology; Sex reversal; Steroid hormones; Steroidogenesis; Steroidogenic Factor 1; Steroids - metabolism; Trans-Activators - physiology; Transcription factors; Transcription Factors - metabolism; Transcription Factors - physiology; Transcription, Genetic; Vertebrates: endocrinology; WT1 Proteins - physiology; Development; Adrenal cortex
• ISSN: 0013-7227; 1945-7170
• DOI: 10.1210/en.2004-1385

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.