[Genetic variant analysis of a pedigree affected with lymphedema-distichiasis syndrome]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2020 Apr 10;37(4):434-437. doi: 10.3760/cma.j.issn.1003-9406.2020.04.017.
[Article in Chinese]

Abstract

Objective: To analyze FOXC2 gene variant in a family affected with lymphodema-distichiasis syndrome (LDS).

Methods: Peripheral blood samples were collected for the extraction of DNA and protein. Whole-exome sequencing was carried out to detect variants in the proband. Suspected variant was validated by Sanger sequencing. Western blotting was used to detect changes in protein expression.

Results: The proband and his mother were both found to carry a heterozygous nonsense variant c.177C>G (p.Tyr59X) of the FOXC2 gene, which was previously unreported. Down-regulated expression of FOXC2 was detected by Western blotting. Prenatal ultrasonography of the fetus indicated increased nuchal thickness. Amniocentesis was performed at 21+1 weeks of pregnancy, genetic testing suggested that the fetus also carried the c.177C>G variant.

Conclusion: The patients' condition may be attributed to the heterozygous nonsense variant c.177C>G of the FOXC2 gene, which resulted in a significant decrease in FOXC2 expression. Increased nuchal thickness may also be related with decreased FOXC2 expression. Above finding has expanded the variant spectrum of the FOXC2 gene.

Publication types

  • Case Reports

MeSH terms

  • Codon, Nonsense
  • Eyelashes / abnormalities*
  • Female
  • Forkhead Transcription Factors / genetics*
  • Forkhead Transcription Factors / metabolism
  • Gene Expression
  • Genetic Testing
  • Genetic Variation
  • Humans
  • Lymphedema / genetics*
  • Pedigree*
  • Pregnancy
  • Prenatal Diagnosis

Substances

  • Codon, Nonsense
  • Forkhead Transcription Factors
  • mesenchyme fork head 1 protein

Supplementary concepts

  • Lymphedema distichiasis syndrome