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Fragile X-related protein 1 (FXR1P) is a member of the FXR gene family, which also includes fragile X mental retardation protein and fragile X-related protein 2 (FXR2P). To understand the functions of FXR1P, we screened FXR1P-interacting proteins using a yeast two-hybrid system. FXR1P was fused to pGBKT7 and used as the bait to screen a human fetal brain cDNA library. This screening revealed 10 FXR1P-interacting proteins including FTH1. FTH1 encodes Homo sapiens ferritin,heavy polypeptide 1. The interaction between FXR1P and FTH1 was confirmed by retesting in yeast using both a β-galactosidase assay and growth studies on selective media. A co-immunoprecipitation assay in mammalian cells further confirmed the FXR1P/FTH1 interaction.Moreover, the results revealed that FTH1 colocalized with FXR1P in the cytoplasm around the nucleus in mammalian cells. The present findings suggest that FXR1P plays an important role in iron metabolism in the brain by interacting with FTH1. This provides clues for elucidating the relationship between FXR1P function and fragile X syndrome.