Work

Steroid 21-hydroxylase expression and activity in human lymphocytes

Year: 1997

Type: article

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-hydroxyprogesterone 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.

Source: Molecular and Cellular Endocrinology

DOI: 10.1016/s0303-7207(96)03997-4

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

Institutions: North Shore University Hospital, New York University, The University of Texas Southwestern Medical Center, Northwell Health

Language: English

Topic: Sexual Differentiation and Disorders

Subfield: Endocrinology, Diabetes and Metabolism

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $3,510