Endocrine Abstracts

The adrenogonadal primordium develops from a thickening of the coelomic epithelium covering the urogenital ridge. After segregation of the primordium into the bipotential gonad and the adrenocortical primordium, the cortex is encapsulated by mesenchymal cells and infiltrated by migrating sympathoadrenal cells, which form the medulla. We have shown that the cell fate regulator sonic hedgehog (Shh) is not required for the formation of the adrenocortical primordium but is require...

Introduction: Bardet-Biedl Syndrome (BBS) is a rare, autosomal recessive ciliopathy, with a prevalence of 1 in 100,000–160,000, caused by mutations across >20 known genes encoding for proteins responsible for primary cilium/basal body complex integrity. Endocrinopathies associated with BBS include hypogonadism, hypothyroidism, and the metabolic complications of obesity. The endocrine characteristics of a large adult BBS cohort have been reported; howe...