Minireview: transcriptional regulation of adrenocortical development

Endocrinology. 2005 Mar;146(3):1018-24. doi: 10.1210/en.2004-1385. Epub 2004 Dec 16.

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.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex / embryology*
  • Adrenal Cortex / physiology*
  • Adrenal Glands / embryology*
  • Adrenal Glands / pathology
  • Animals
  • DAX-1 Orphan Nuclear Receptor
  • DNA-Binding Proteins / physiology
  • Epinephrine / metabolism
  • Gene Expression Regulation, Developmental*
  • Homeodomain Proteins
  • Humans
  • Mesoderm / metabolism
  • Mice
  • Models, Biological
  • Norepinephrine / metabolism
  • Pre-B-Cell Leukemia Transcription Factor 1
  • Proto-Oncogene Proteins / physiology
  • Receptors, Cytoplasmic and Nuclear
  • Receptors, Retinoic Acid / physiology
  • Repressor Proteins / physiology
  • Steroidogenic Factor 1
  • Steroids / metabolism
  • Trans-Activators / physiology
  • Transcription Factors / metabolism
  • Transcription Factors / physiology
  • Transcription, Genetic*
  • WT1 Proteins / physiology

Substances

  • CITED2 protein, human
  • DAX-1 Orphan Nuclear Receptor
  • DNA-Binding Proteins
  • Homeodomain Proteins
  • NR0B1 protein, human
  • NR5A1 protein, human
  • Nr0b1 protein, mouse
  • Pre-B-Cell Leukemia Transcription Factor 1
  • Proto-Oncogene Proteins
  • Receptors, Cytoplasmic and Nuclear
  • Receptors, Retinoic Acid
  • Repressor Proteins
  • Steroidogenic Factor 1
  • Steroids
  • Trans-Activators
  • Transcription Factors
  • WT1 Proteins
  • PBX1 protein, human
  • steroidogenic factor 1, mouse
  • Norepinephrine
  • Epinephrine