Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
1934195 | Biochemical and Biophysical Research Communications | 2008 | 6 Pages |
Abstract
The Aristaless-related homeobox (Arx) is mutated in patients with X-linked mental retardation and a range of other neurological diseases. The molecular consequences of these mutations are unclear. Here, we show that two disease-associated mutations disrupt the function of Arx as a transcriptional repressor. We found that Arx contains two independent repression domains: an N-terminal octapeptide motif/engrailed homology domain and a novel domain located in the C-terminus. The octapeptide motif functions through interaction with members of the Groucho family of co-repressors. The C-terminal domain functions through interaction with C-terminal binding protein (CtBP).
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Authors
Amy N. Fullenkamp, Heithem M. El-Hodiri,