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Fragile X syndrome is a common form of mental retardation caused by the absence of the FMR1 protein, FMRP.  Fmr1  knockout mice exhibit a phenotype with some similarities to humans, such as macro-orchidism and behavioral abnormalities. Two homologs of FMRP have been identified, FXR1P and FXR2P. These proteins show high sequence similarity, including all functional domains identified in FMRP, such as RNA binding domains. They have an overlap in tissue distribution to that of FMRP. Interactions between the three FXR proteins have also been described. FXR2P shows high expression in brain and testis, like FMRP. To study the function of FXR2P, we generated an  Fxr2  knockout mouse model. No pathological differences between knockout and wild-type mice were found in brain or testis. Given the behavioral phenotype in fragile X patients and the phenotype previously reported for the  Fmr1  knockout mouse, we performed a thorough evaluation of the  Fxr2  knockout phenotype using a behavioral test battery.  Fxr2  knockout mice were hyperactive (i.e. traveled a greater distance, spent more time moving and moved faster) in the open-field test, impaired on the rotarod test, had reduced levels of prepulse inhibition, displayed less contextual conditioned fear, impaired at locating the hidden platform in the Morris water task and were less sensitive to a heat stimulus. Interestingly, there are some behavioral phenotypes in  Fxr2  knockout mice which are similar to those observed in  Fmr1  knockout mice, but there are also some different behavioral abnormalities that are only observed in the  Fxr2  mutant mice. The findings implicate a role for  Fxr2  in central nervous system function.

Knockout mouse model for Fxr2 : a model for mental retardation