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

24 Scopus citations

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

Keywords

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

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Endocrinology

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