Stains & molecular markers
3 beta hydroxysteroid dehydrogenase / HSD3B1 and HSD3B2

Topic Completed: 1 July 2013

Minor changes: 11 December 2020

Copyright: 2003-2022,, Inc.

PubMed Search: HSD3B1 and HSD3B2

Nat Pernick, M.D.
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Cite this page: Pernick, N. 3 beta hydroxysteroid dehydrogenase / HSD3B1 and HSD3B2. website. Accessed January 23rd, 2022.
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)
Diagrams / tables

Images hosted on other servers:

Steroid synthesis chart

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

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gland zona
(fig B)

Placental villi

Positive staining - normal
Positive staining - disease
Negative staining
  • Normal seminiferous tubules, fetal adrenal gland
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