Table of Contents
Definition / general | Diagrams / tables | Uses by pathologists | Microscopic (histologic) images | Positive staining - normal | Positive staining - disease | Negative stainingCite this page: 3 beta hydroxysteroid dehydrogenase / HSD3B1 and HSD3B2. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/stains3beta.html. Accessed July 12th, 2017.
Definition / general
- Critical enzyme in biosynthesis of all steroid hormones; catalyzes conversions of progesterone from pregnenolone, 17-hydroxyprogesterone from 17-hydroxypregnenolone and androstenedione from dehydroepiandrosterone (Wikipedia)
- Also called hydroxyl-δ-5-steroid dehydrogenase
- Isomerase 1 (HSD3B1) localizes generally to placenta and nonsteroidogenic tissue; isomerase 2 (HSD3B2) generallly localizes to adrenal gland, gonads and steroidogenic tissue
- Located on inner mitochondrial membrane; acts as both a dehydrogenase and an isomerase, due to a conformational change (Biochemistry 2011;50:11015)
- Transcription in adrenal gland regulated by farnesoid X receptor (Mol Cell Endocrinol 2009;299:153)
- Mutations cause rare form of congenital adrenal hyperplasia (Horm Res Paediatr 2012;77:334, Rev Med Liege 2004;59:485, Gene 2012;503:215)
Uses by pathologists
- Identify adenocortical tissue origin (Arch Pathol Lab Med 2002;126:1118)
- Note: fetal adrenal gland lacks 3 beta hydroxysteroid dehydrogenase
- Confirm trophoblastic origin
Microscopic (histologic) images
Positive staining - normal
- Adrenocortical tissue, placental intermediate trophoblast and syncytiotrophoblast (Am J Surg Pathol 2008;32:236); testicular Leydig cells
Positive staining - disease
- Trophoblastic tumors (HSD3B1 - Am J Surg Pathol 2008;32:236, Int J Gynecol Cancer 2013;23:343)
Negative staining
- Normal seminiferous tubules, fetal adrenal gland




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