CONTEXT Lipoid congenital adrenal hyperplasia is a severe disorder of adrenal and gonadal steroidogenesis caused by mutations in the steroidogenic acute regulatory protein (StAR). Affected children typically present with life-threatening adrenal insufficiency in early infancy due to a failure of glucocorticoid (cortisol) and mineralocorticoid (aldosterone) biosynthesis, and 46,XY genetic males ...

The trophic peptide hormone adrenocorticotropic (ACTH) stimulates steroid hormone biosynthesis evoking both a rapid, acute response and a long-term, chronic response, via the activation of cAMP/protein kinase A (PKA) signaling. The acute response is initiated by the mobilization of cholesterol from lipid stores and its delivery to the inner mitochondrial membrane, a process that is mediated by ...

Primary ovarian insufficiency (POI) is characterized by the loss of ovarian function before the age of 40 in humans. Although most cases of POI are idiopathic, many are familial, underlying a genetic origin of the disease. Mutations in genes involved in the control of steroidogenesis, such as NR5A1 (SF-1, Steroidogenic Factor 1), CYP17, CYP19A1 (aromatase), StAR (Steroidogenic Acute Regulatory)...

Background: Methotrexate (MTX) is used as an antineoplastic drug in the treatment of various cancers and non-cancerous diseases. Ovarian ablation is considered to be effective in delaying recurrence of cancer and increasing survival in young women. When added to chemotherapy, it is less clear that this technique is effective. Uniqueness of the present study is that we used ovariectomized (OVX) ...

Background: Methotrexate (MTX) is used as an antineoplastic drug in the treatment of various cancers and non-cancerous diseases. Ovarian ablation is considered to be effective in delaying recurrence of cancer and increasing survival in young women. When added to chemotherapy, it is less clear that this technique is effective. Uniqueness of the present study is that we used ovariectomized (OVX) ...

Adrenocorticotropin hormone (ACTH) produced by the anterior pituitary stimulates glucocorticoid synthesis by the adrenal cortex. The first step in glucocorticoid synthesis is the delivery of cholesterol to the mitochondrial matrix where the first enzymatic reaction in the steroid hormone biosynthetic pathway occurs. A key response of adrenal cells to ACTH is activation of the cAMP-protein kinas...