A recurrent mutation in MED12 leading to R961W causes Opitz-Kaveggia syndrome

Hiba Risheg, John M. Graham, Robin D. Clark, R. Curtis Rogers, John M. Opitz, John B. Moeschler, Andreas P. Peiffer, Melanie May, Sumy M. Joseph, Julie R. Jones, Roger E. Stevenson, Charles E. Schwartz, Michael J. Friez

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Abstract

Opitz-Kaveggia syndrome (also known as FG syndrome) is an X-linked disorder characterized by mental retardation, relative macrocephaly, hypotonia and constipation. We report here that the original family for whom the condition is named and five other families have a recurrent mutation (2881C>T, leading to R961W) in MED12 (also called TRAP230 or HOPA), a gene located at Xq13 that functions as a thyroid receptor-associated protein in the Mediator complex.

Original languageEnglish
Pages (from-to)451-453
Number of pages3
JournalNature Genetics
Volume39
Issue number4
DOIs
StatePublished - Apr 2007

ASJC Scopus Subject Areas

  • Genetics

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