CYP11B1 variants influence skeletal maturation via alternative splicing

Commun Biol. 2021 Nov 9;4(1):1274. doi: 10.1038/s42003-021-02774-y.

Abstract

We performed genome-wide association study meta-analysis to identify genetic determinants of skeletal age (SA) deviating in multiple growth disorders. The joint meta-analysis (N = 4557) in two multiethnic cohorts of school-aged children identified one locus, CYP11B1 (expression confined to the adrenal gland), robustly associated with SA (rs6471570-A; β = 0.14; P = 6.2 × 10-12). rs6410 (a synonymous variant in the first exon of CYP11B1 in high LD with rs6471570), was prioritized for functional follow-up being second most significant and the one closest to the first intron-exon boundary. In 208 adrenal RNA-seq samples from GTEx, C-allele of rs6410 was associated with intron 3 retention (P = 8.11 × 10-40), exon 4 inclusion (P = 4.29 × 10-34), and decreased exon 3 and 5 splicing (P = 7.85 × 10-43), replicated using RT-PCR in 15 adrenal samples. As CYP11B1 encodes 11-β-hydroxylase, involved in adrenal glucocorticoid and mineralocorticoid biosynthesis, our findings highlight the role of adrenal steroidogenesis in SA in healthy children, suggesting alternative splicing as a likely underlying mechanism.

MeSH terms

  • Age Determination by Skeleton
  • Alternative Splicing*
  • Bone Development / genetics*
  • Child
  • Female
  • Humans
  • Male
  • Steroid 11-beta-Hydroxylase / genetics*
  • Steroid 11-beta-Hydroxylase / metabolism

Substances

  • Steroid 11-beta-Hydroxylase