Steroid 21-hydroxylase expression and activity in human lymphocytes
Open accessAbstract
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.
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Low expression of CYP21A2 during the second trimester partially explains the high incidence of false-positives in newborn screens for CAH in premature infants.
“Low expression of CYP21A2 during the second trimester partially explains the high incidence of false-positives in newborn screens for CAH in premature infants (see section IV.A)(159).”
From Congenital Adrenal Hyperplasia—Current Insights in Pathophysiology, Diagnostics, and Management (2022) · cites this paper as [159]
Section 3.1 reports CYP21 expression in cultured lymphoid cells, including cells from CAH patients, but does not examine expression during the second trimester. Searches for premature infants, newborn screening, and false positives found no passage addressing the proposed explanation. (LLM, conf=0.76)
Experimental — accuracy not yet measuredPremature, sick, or stressed infants tend to have higher levels of 17OHP than term infants; preterm infants have a functional deficiency of several adrenal steroidogenic enzymes with a nadir in function at 29 weeks of gestation.
“First, premature, sick, or stressed infants tend to have higher levels of 17OHP than term infants; as studied by high-performance liquid chromatography, preterm infants have a functional deficiency of several adrenal steroidogenic enzymes with a nadir in function at 29 weeks of gestation (159).”
From Congenital Adrenal Hyperplasia—Current Insights in Pathophysiology, Diagnostics, and Management (2022) · cites this paper as [159]
The Methods (§2.5) describe thin-layer chromatography of steroids incubated with lymphocytes, and the Results (§3.2) report 21-hydroxylase activity in those cells. Our copy does not address 17OHP levels in premature, sick, or stressed infants, deficiencies of several adrenal enzymes in preterm infants, or a functional nadir at 29 weeks; searches for those terms and for high-performance liquid chromatography found no supporting passage. Page numbers were unavailable. (LLM, conf=0.76)
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References 35 · Cited by 28
Research works printed but not linked: 7
- Speiser, P.W. (1995). In: Principles and Practice of Endocrinol- ogy and Metabolism (Becker, K., ed.), pp. 686 -695, J.B. Lippin- cott, New York.
- Wedell, A., Ritzen, E.M., Haglund-Stengler, B. and Luthman, H. (1992) Proc. Natl. Acad. Sci. USA 89, 7232 -7236.
- White, P.C., Vitek, A., Dupont, B. and New, M.I. (1988) Proc. Natl. Acad. Sci. USA 85, 4436 -4440.
- Donohoue, P.A., Jospe, N., Migeon, C.J. and Van Dop, C. (1989) [published erratum appears in Genomics 1990 Feb, 6(2):392] Genomics 5, 397 -406.
- White, P.C., New, M.I. and Dupont, B. (1984) Proc. Natl. Acad. Sci. USA. 81, 7505 -7509.
- Amor, M., Parker, K.L., Globerman, H., New, M.I. and White, P.C. (1988) Proc. Natl. Acad. Sci. USA 85, 1600-1604.
- White, P.C., Grossberger, D., Onufer, B.J., Chaplin, D.D., New, M.I., Dupont, B. and Strominger, J.L. (1985) Proc. Natl. Acad. Sci. USA 82, 1089 -1093.
- Authors
- Zhou, Z., Agarwal, V. R., Dixit, N., White, P., & Speiser, P. W.
- Affiliations
- North Shore University Hospital (US)
- The University of Texas Southwestern Medical Center (US)
- Northwell Health (US)
- Corresponding author
- Speiser, Phyllis W
- Venue
- Molecular and Cellular Endocrinology
- Publisher
- Elsevier BV
- Published
- 1997-03
- Volume
- 127
- Issue
- 1
- Pages
- 11-18
- ISSN
- 0303-7207 (ISSN-L)
- OpenAlex
- W1977681440
- Cited by
- 28
- References
- 35
- Citations by year
- 2021: 1 · 2020: 1 · 2015: 1 · 2014: 1
- FWCI
- 0.47
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Molecular and Cellular Endocrinology (publishedVersion, cc-by-nc-nd)
- PubMed (publishedVersion)
- Funders
- Eli Lilly and Company
- Genentech
- National Institute of Diabetes and Digestive and Kidney Diseases (DK-37867)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (HD 00072)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Hormonal Regulation and Hypertension
- Keywords
- 21-hydroxylase, CYP21, congenital adrenal hyperplasia, B cells, 17-hydroxyprogesterone, 11-deoxycortisol, deoxycorticosterone
- MeSH terms
- Adrenal Hyperplasia, Congenital, B-Lymphocytes, Cell Line, Genetic Markers, Humans, Phenotype, Steroid 21-Hydroxylase, Gene Expression Regulation, Enzymologic, Polymerase Chain Reaction, Gene Deletion, DNA Primers
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