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A novel variant in MED12 gene: Further delineation of phenotype.

Dhanya Lakshmi NarayananShubha R Phadke
Published in: American journal of medical genetics. Part A (2017)
MED12 is a multiprotein mediator complex, which has a role in cell growth and differentiation and has been implicated in three distinct X-linked intellectual disability syndromes with distinctive clinical features. These include Opitz-Kaveggia syndrome (FG syndrome), Lujan syndrome, and X-linked Ohdo syndrome. Recently MED12 variants have been implicated in isolated X-linked intellectual disability. We describe a 5-year-old male patient with intellectual disability and facial dysmorphism and a novel variant in MED12 gene identified by Whole Exome Sequencing. His dysmorphic facial features are distinct from the previously described phenotypes. With a strong genotype-phenotype correlation that is already known for MED12, this could be a new phenotype linked to MED12, thus expanding the phenotypic spectrum of MED12-related disorders.
Keyphrases
  • intellectual disability
  • autism spectrum disorder
  • case report
  • genome wide
  • dna methylation
  • transcription factor
  • soft tissue