Common genetic variants of the human steroid 21-hydroxylase gene (CYP21A2) are related to differences in circulating hormone levels

PLoS One. 2014 Sep 11;9(9):e107244. doi: 10.1371/journal.pone.0107244. eCollection 2014.

Abstract

Purpose: Systematic evaluation of the potential relationship between the common genetic variants of CYP21A2 and hormone levels.

Methods: The relationships of CYP21A2 intron 2 polymorphisms and haplotypes with diverse baseline and stimulated blood hormone levels were studied in 106 subjects with non-functioning adrenal incidentaloma (NFAI). The rationale for using NFAI subjects is dual: i) their baseline hormone profiles do not differ from those of healthy subjects and ii) hormone levels after stimulation tests are available.

Results: The carriers (N = 27) of a well-defined CYP21A2 haplotype cluster (c5) had significantly elevated levels of cortisol (p = 0.0110), and 17-hydroxyprogesterone (p = 0.0001) after ACTH stimulation, and 11-deoxycortisol after metyrapone administration (p = 0.0017), but the hormone values were in normal ranges. In addition, the carriers (N = 33) of the C allele of the rs6462 polymorphism had a higher baseline aldosterone level (p = 0.0006). The prevalence of these genetic variants of CYP21A2 did not differ between NFAI and healthy subjects.

Conclusions: The common CYP21A2 variants presumably exert the same effect on hormone levels in the healthy and disease-affected populations. Therefore, they may contribute to complex diseases such as some cardiovascular diseases, and may influence the genotype-phenotype correlation in patients with congenital adrenal hyperplasia (CAH) including the individual need for hormone substitution.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • 17-alpha-Hydroxyprogesterone / blood*
  • Adenoma / blood
  • Adenoma / genetics
  • Adrenal Gland Neoplasms / genetics
  • Adrenal Gland Neoplasms / metabolism
  • Adrenal Hyperplasia, Congenital / blood
  • Adrenal Hyperplasia, Congenital / genetics
  • Adult
  • Case-Control Studies
  • Female
  • Gene Frequency
  • Haplotypes
  • Heterozygote
  • Humans
  • Hydrocortisone / blood*
  • Linkage Disequilibrium
  • Male
  • Middle Aged
  • Polymorphism, Genetic
  • Ranitidine
  • Retrospective Studies
  • Steroid 21-Hydroxylase / genetics*

Substances

  • 17-alpha-Hydroxyprogesterone
  • Ranitidine
  • CYP21A2 protein, human
  • Steroid 21-Hydroxylase
  • Hydrocortisone

Supplementary concepts

  • Adrenal incidentaloma

Grants and funding

Hungarian Scientific Research Fund (OTKA, PD100648 (AP)) Technology Innovation Fund, National Developmental Agency (KTIA-AIK-2012-12-1-0010). AP is the recipient of a “Lendület” grant from the Hungarian Academy of Sciences. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.