The past several years have seen remarkable growth in our understanding of the molecular processes underlying fragile X syndrome (FXS). Many studies have provided new insights into the regulation of Fmr1 gene expression and the potential function of its protein product. It is now known that the promoter elements modulating Fmr1 transcription involve a complex array of both cis and trans factors. Moreover, recent studies of epigenetic modification of chromatin have provided novel clues to unlocking the mysteries behind the regulation of Fmr1 expression. Here, we review the latest findings on the regulation of Fmr1 transcription.
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