Quantitative PCR from human genomic DNA: The determination of gene copy numbers for congenital adrenal hyperplasia and RCCX copy number variation

PLoS One. 2022 Dec 1;17(12):e0277299. doi: 10.1371/journal.pone.0277299. eCollection 2022.

Abstract

Quantitative PCR (qPCR) is used for the determination of gene copy number (GCN). GCNs contribute to human disorders, and characterize copy number variation (CNV). The single laboratory method validations of duplex qPCR assays with hydrolysis probes on CYP21A1P and CYP21A2 genes, residing a CNV (RCCX CNV) and related to congenital adrenal hyperplasia, were performed using 46 human genomic DNA samples. We also performed the verifications on 5 qPCR assays for the genetic elements of RCCX CNV; C4A, C4B, CNV breakpoint, HERV-K(C4) CNV deletion and insertion alleles. Precision of each qPCR assay was under 1.01 CV%. Accuracy (relative error) ranged from 4.96±4.08% to 9.91±8.93%. Accuracy was not tightly linked to precision, but was significantly correlated with the efficiency of normalization using the RPPH1 internal reference gene (Spearman's ρ: 0.793-0.940, p>0.0001), ambiguity (ρ = 0.671, p = 0.029) and misclassification (ρ = 0.769, p = 0.009). A strong genomic matrix effect was observed, and target-singleplex (one target gene in one assay) qPCR was able to appropriately differentiate 2 GCN from 3 GCN at best. The analysis of all GCNs from the 7 qPCR assays using a multiplex approach increased the resolution of differentiation, and produced 98% of GCNs unambiguously, and all of which were in 100% concordance with GCNs measured by Southern blot, MLPA and aCGH. We conclude that the use of an internal (in one assay with the target gene) reference gene, the use of allele-specific primers or probes, and the multiplex approach (in one assay or different assays) are crucial for GCN determination using qPCR or other methods.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenal Hyperplasia, Congenital* / genetics
  • DNA
  • DNA Copy Number Variations* / genetics
  • Gene Dosage
  • Genomics
  • Humans
  • Real-Time Polymerase Chain Reaction
  • Steroid 21-Hydroxylase / genetics

Substances

  • DNA
  • CYP21A2 protein, human
  • Steroid 21-Hydroxylase

Grants and funding

The current research was supported by Semmelweis Science and Innovation Fund to MD (STIA-KF-17) and Hungarian Scientific Research Fund to AP (K125231). MD was supported by Janos Bolyai Research Scholarship from the Hungarian Academy of Sciences, and the UNKP-19-4 New National Excellence Program of the Ministry for Innovation and Technology. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.