Fmr1-KO mice contain a neomycin resistance cassette substituting exon 5 of the fragile X mental retardation syndrome 1 (Fmr1) gene. The knockdown causes an increase in the number of CGG repeats that lead to hypermethylation of the Fmr1 gene and therefore inhibiting FMR protein production. The mouse is a well-known model of Fragile X Syndrome, a monogenic cause of autism spectrum disorders and one of the most common inherited forms of intellectual disability.
Mice present core and secondary phenotypic traits of Fragile X Syndrome such as hyperactivity (B), altered anxiety levels (C) as well as repetitive behavior (D)
Figure: Activity, hyperactivity, anxiety, and repetitive behavior of male Fmr1-KO mice at the age of 7 weeks. Activity (A) and hyperactivity (B) measured in the open field test, time spent in open arms of the open field test (C), and time spent grooming in the auto-grooming test (D) of Fmr1-KO compared to C57BL/6JRj mice. n = 15 per group. Unpaired t-test; mean + SEM; **p<0.01.
Our results and that of others (Bakker et al., 1994; Mineur et al., 2002) show that Fmr1-Ko mice display the most common symptoms of Fragile X Syndrome, such as increased activity and hyperactivity, altered anxiety as well as increase repetitive behavior and are thus a valuable model for Fragile X Syndrome research.
QPS Neuropharmacology offers a custom-tailored study design for this model and we are flexible to accommodate to your special interest. We are also happy to advice you and propose study designs. A typical turnaround time from agreement to the study plan to the final report is about 3 months. Animals will be purchased right after your order. Animals of all age groups are typically available without any delay. Fmr1-KO mice show relevant features of Fragile X Syndrome already at young age. This allows for extraordinarily fast turn-around times. Furthermore, C57BL/6 mice are available as control animals needed for proper study design.
We would be happy to test your compounds in the Fmr1-KO mouse model! The most common readouts are:
- Activity / Hyperactivity
- Repetitive behavior
- Social behavior
Looking for something else? Please contact us!