Abstract

Fragile X syndrome, the most common form of inherited mental retardation in humans, affects about 1 in 3000 males and 1 in 5000 females. It is caused by the loss of expression of the fragile X mental retardation protein (FMRP) due to a CGG trinucleotide repeat expansion in the 5’-untranslated region (UTR) of the fragile x mental retardation-1 (fmr1) gene. FMRP has been shown to use its arginine-glycine-glycine (RGG) box RNA binding domain to bind with high affinity and specificity to G quadruplex forming mRNA sequences.

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