[Wiedemann-Steiner syndrome due to novel nonsense variant of KMT2A gene in a case]

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2021 Feb 10;38(2):138-140. doi: 10.3760/cma.j.cn511374-20200122-00046.
[Article in Chinese]

Abstract

Objective: To explore the genetic basis for a child with unexplained global developmental delay (GDD), seizure, and facial deformity.

Methods: Whole exome sequencing (WES) was carried out for the patient. Candidate variants were verified by Sanger sequencing of the patient and his parents.

Results: WES revealed that the patient has carried a previously unreported de novo heterozygous nonsense c.4906C>T (p.Arg1636Ter) variant of the KMT2A gene, Based on the American College of Medical Genetics and Genomics standards and guidelines, the c.4906C>T variant of KMT2A gene was predicted to be pathogenic (PVS1+ PS2+ PM2+PP3).

Conclusion: The heterozygous nonsense c.4906C>T (p.Arg1636Ter) variant of the KMT2A gene probably underlay the disease in the child. Above finding has enriched the spectrum of pathogenic variants of the KMT2A gene.

MeSH terms

  • Abnormalities, Multiple / genetics*
  • Child
  • Histone-Lysine N-Methyltransferase / genetics*
  • Humans
  • Intellectual Disability / genetics*
  • Male
  • Myeloid-Lymphoid Leukemia Protein / genetics*
  • Syndrome

Substances

  • KMT2A protein, human
  • Myeloid-Lymphoid Leukemia Protein
  • Histone-Lysine N-Methyltransferase