CGG Triplet Repeat Binding Protein 1 (CGGBP1)

CGGBP1 influences expression of the FMR1 gene, which is associated with the fragile X mental retardation syndrome, by specifically interacting with the 5-prime (CGG)n-3-prime repeat in its 5-prime UTR.The predicted 166-amino acid protein contains a putative nuclear localization signal. Northern blot analysis revealed that p20-CGGBP is expressed as a 1.2-kb transcript in HeLa cells. Dot blot hybridization indicated that this mRNA is expressed in a number of human tissues.CGGBP gene contains 4 exons, with the entire open reading frame located within exon 4. The promoter region contains CAAT boxes and several Sp1-binding sites.CGGBP1 bound to 5-prime-(CGG)n-3-prime repeats in vitro with n equal to or greater than 5. It also bound interrupted repeats.