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Structure and functions

Fragile X messenger ribonucleoprotein (FMRP) is encoded by the fragile X messenger ribonucleoprotein 1 (FMR1) gene, located in the X chromossome, associated with the fragile X syndrome (FXS), Fragile X Tremor/Ataxia Syndrome (FXTAS) and Premature Ovarian Failure (POF1). FMRP functions as a synaptic regulator by binding to mRNAs and inhibiting its translation, therefore regulating the synthesis of proteins in the synapse. It is also a RNA binding protein, which is reponsible for the transportation of mRNAs to cytoplam. The FMRP can also bind to its own FMR1 transcripts, possibly a self-regulatory mechanism. The RGG motif bind to G-quadruplexes, secondary structures formed in some RNAs. The structure being represented on the right represents the FMRP RGG motif and the G-quadruplex secondary structure in the RNA. The protein structure was obtained by X-ray diffraction with a 3 Å resolution [1]

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Daniel Key Takemoto

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