[Analysis of IQSEC2 gene variant in a child with X-linked mental retardation]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2022 Apr 10;39(4):421-424. doi: 10.3760/cma.j.cn511374-20201030-00765.
[Article in Chinese]

Abstract

Objective: To analyze the clinical phenotype and genetic variants of a child with X-linked mental retardation caused by IQSEC2 gene mutation, and provide reference for the diagnosis of the disease.

Methods: The child was subjected to next generation sequencing (NGS), and the diagnosis was made by taking consideration of her clinical characteristics.

Results: The child has presented with global developmental delay, particularly in fine motor skill and language development, in addition with intellectual disability. Genetic testing revealed that she has harbored a heterozygous c.1861dup variant of the IQSEC2 gene, which was not detected in either parent.

Conclusion: The de novo c.186ldup variant of the IQSEC2 gene probably underlay the X-linked mental retardation in this child. Above finding has, expanded the spectrum of IQSEC2 gene mutations and provide a basis for the diagnosis of similar cases.

MeSH terms

  • Female
  • Guanine Nucleotide Exchange Factors / genetics
  • Heterozygote
  • Humans
  • Intellectual Disability* / genetics
  • Mental Retardation, X-Linked* / genetics
  • Mutation
  • Phenotype

Substances

  • Guanine Nucleotide Exchange Factors
  • IQSEC2 protein, human