Steroid 21-hydroxylase expression and activity in human lymphocytes

Zhifeng Zhou, Veena R. Agarwal, Naznin Dixit, Perrin White, Phyllis W. Speiser

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Steroid 21-hydroxylase encoded by CYP21 is expressed in adrenal cortex. Mutations in CYP21 cause potentially lethal congenital adrenal hyperplasia (CAH). Earlier observations suggested alternative sources of 21-hydroxylase activity, although its genetic source remains unclear. We found a novel source of CYP21 expression in normal human cultured B lymphocytes. The quantity of 21-hydroxylase transcript was reduced in B cell lines of CAH subjects compared with that in normal B-lymphoblastoid cells. No CYP21 transcript was detected in lymphocytes from a CAH patient with homozygous CYP21 deletion. Cultured lymphoid cells, including those carrying homozygous CYP21 deletion, and peripheral blood leukocytes converted both 17-hydroxy-progesterone to 11-deoxycortisol and progesterone to deoxycorticosterone. We conclude that lymphocytes express CYP21, but also possess a 21-hydroxylase distinct from CYP21. Activity of this isozyme may partially compensate for severe adrenal 21-hydroxylase deficiency.

Original languageEnglish (US)
Pages (from-to)11-18
Number of pages8
JournalMolecular and Cellular Endocrinology
Volume127
Issue number1
DOIs
StatePublished - Mar 14 1997

Fingerprint

Steroid 21-Hydroxylase
Lymphocytes
Human Activities
Congenital Adrenal Hyperplasia
Progesterone
B-Lymphocytes
Cortodoxone
Desoxycorticosterone
Adrenal Cortex
Isoenzymes
Cultured Cells
Leukocytes
Cell Line
Blood
Mutation
Cells

Keywords

  • Adrenal hyperplasia
  • Congenital
  • Isozymes
  • Lymphocytes
  • Phenotype
  • Steroid 21-monooxygenase

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism

Cite this

Steroid 21-hydroxylase expression and activity in human lymphocytes. / Zhou, Zhifeng; Agarwal, Veena R.; Dixit, Naznin; White, Perrin; Speiser, Phyllis W.

In: Molecular and Cellular Endocrinology, Vol. 127, No. 1, 14.03.1997, p. 11-18.

Research output: Contribution to journalArticle

Zhou, Zhifeng ; Agarwal, Veena R. ; Dixit, Naznin ; White, Perrin ; Speiser, Phyllis W. / Steroid 21-hydroxylase expression and activity in human lymphocytes. In: Molecular and Cellular Endocrinology. 1997 ; Vol. 127, No. 1. pp. 11-18.
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