Mutations in ZP4 are associated with abnormal zona pellucida and female infertility

J Clin Pathol. 2022 Mar;75(3):201-204. doi: 10.1136/jclinpath-2020-207170. Epub 2021 Jan 18.

Abstract

Background: The zona pellucida (ZP) of human oocytes plays essential protective roles in sperm-egg interactions during fertilisation and embryo development. ZP4-null female rabbits exhibit a thin and irregular ZP, which severely impairs embryo development and fertility. However, the effects of ZP4 defect on human female reproduction remain unknown.

Methods and results: We performed whole-exome sequencing in 26 female patients with abnormal (thin and irregular) ZP and identified heterozygous variants in ZP4 (OMIM: 613514) from 3 patients (approximately 11%). No ZP4 variant was found in the 30 control women with proven fertility. We constructed ZP4-mutated plasmids and found that the variants reduced the secretion of ZP4 in vitro. Lower suction pressure facilitated egg retrieval, and intracytoplasmic sperm injection (ICSI) was a desirable treatment for ZP4-mutated patients with abnormal ZP.

Conclusions: We identified ZP4 as a novel gene for human abnormal ZP and found that lower suction pressure and ICSI are efficient treatment strategies.

Keywords: DNA; cell biology; molecular; ovary; pathology.

MeSH terms

  • Embryonic Development
  • Exome Sequencing
  • Female
  • Fertility
  • Gene Expression
  • Humans
  • Infertility, Female / genetics*
  • Infertility, Female / pathology
  • Mutation
  • Zona Pellucida / pathology
  • Zona Pellucida Glycoproteins / genetics*
  • Zona Pellucida Glycoproteins / metabolism

Substances

  • ZP4 protein, human
  • Zona Pellucida Glycoproteins