Narayanan Dhanya L, Phadke Shubha R
Department of Medical Genetics, Sanjay Gandhi Post Graduate Institute, Lucknow, India.
Am J Med Genet A. 2017 Aug;173(8):2257-2260. doi: 10.1002/ajmg.a.38295. Epub 2017 May 23.
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.
MED12是一种多蛋白中介复合物,在细胞生长和分化中起作用,并且与三种具有独特临床特征的不同X连锁智力障碍综合征有关。这些综合征包括奥皮茨-卡韦吉亚综合征(FG综合征)、卢扬综合征和X连锁奥多综合征。最近,MED12变异体与孤立性X连锁智力障碍有关。我们描述了一名患有智力障碍和面部畸形的5岁男性患者,通过全外显子测序鉴定出MED12基因中的一种新型变异体。他的面部畸形特征与先前描述的表型不同。鉴于MED12已经存在很强的基因型-表型相关性,这可能是一种与MED12相关的新表型,从而扩大了MED12相关疾病的表型谱。