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Steroid biosynthesis

About: Steroid biosynthesis is a research topic. Over the lifetime, 1721 publications have been published within this topic receiving 58977 citations.


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Book ChapterDOI
TL;DR: This chapter briefly describes the clinical and biochemical features of ACTH resistance syndromes and focuses on the recent progress made towards understanding the molecular defect underlying these conditions, in particular the interaction of MC2R and MRAP.
Abstract: Familial glucocorticoid deficiency (FGD) and triple A syndrome belong to a rare group of autosomal recessive disorders characterized by adrenocorticotropin (ACTH) insensitivity Unlike triple A syndrome which presents a range of clinical features, FGD is solely characterized by glucocorticoid deficiency ACTH regulates steroid biosynthesis in the adrenal cortex by exerting its effects via the ACTH receptor (melanocortin- 2 receptor, MC2R) In FGD, mutations in the MC2R account for only approximately 25% of cases (FGD type 1) The inability to express a functional MC2R in non-adrenal cell lines had implied the presence of an adrenal specific accessory factor(s), essential for MC2R expression More recently, this factor was identified as melanocortin receptor accessory protein (MRAP) Mutations in MRAP account for 20% of cases (FGD type 2) Like the receptor activity-modifying proteins (RAMPs) and receptor transporter proteins (RTPs), which are well-characterized accessory proteins for G-protein-coupled receptors (GPCRs), MRAP is a small single transmembrane domain protein MRAP is essential for the functional expression of the MC2R About 55% of FGD cases have no identifiable gene defect, implying the involvement of additional genes This chapter briefly describes the clinical and biochemical features of ACTH resistance syndromes However, we will focus on the recent progress made towards understanding the molecular defect underlying these conditions, in particular the interaction of MC2R and MRAP

31 citations

Journal ArticleDOI
TL;DR: In vitro steroid metabolism was investigated in Xenopus laevis vitellogenic follicles and full-grown follicles, finding that 10 to 50 times more estrogens were found to be associated with follicular envelopes.

31 citations

Journal ArticleDOI
TL;DR: Ginkgolide B (GKB) is a purified component of Ginkgo biloba leaf extracts that reduces the mRNA, protein, and ligand-binding levels of the adrenal peripheral-type benzodiazepine receptor (PBR), a mitochondrial cholesterol-binding protein, leading to decreased corticosteroid synthesis as mentioned in this paper.

31 citations

Journal ArticleDOI
TL;DR: The results indicated that the regressing ovary has lower response to gonadotropic hormone stimulation than the growing ovary and embryonic chicken ovary receptors are more sensitive to LH- like activity than FSH-like activity.

31 citations


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Performance
Metrics
No. of papers in the topic in previous years
YearPapers
202315
202221
2021117
2020109
201975
201860