[Genetic analysis of a case with Pitt-Hopkins syndrome due to variant of TCF4 gene]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2020 Nov 10;37(11):1253-1256. doi: 10.3760/cma.j.cn511374-20190725-00372.
[Article in Chinese]

Abstract

Objective: To explore the genetic basis of a patient presenting with dysmorphism, intellectual disability, psychomotor delay and hypoplasia of corpus callosum by using next generation sequencing.

Methods: Genomic DNA was extracted from peripheral blood samples of the patient and his family members and subjected to exome sequencing. Suspected variants were verified with Sanger sequencing.

Results: The patient was found to carry a heterozygous c.1357delAinsGGA variant in exon 11 of the TCF4 gene, which was verified as de novo by Sanger sequencing. The variant may result in a truncated protein and affect its function.

Conclusion: The heterozygous c.1357delAinsGGA variant the TCF4 gene probably underlies the disease in the proband.

MeSH terms

  • Facies
  • Genetic Testing
  • Humans
  • Hyperventilation / genetics*
  • Intellectual Disability / genetics*
  • Male
  • Transcription Factor 4 / genetics*

Substances

  • TCF4 protein, human
  • Transcription Factor 4

Supplementary concepts

  • Pitt-Hopkins syndrome