Studies in Mouse Models of Fragile X Syndrome
Ben A. Oostra, PhD
Principal Investigator
Femke de Vrij, PhD
Postdoctoral Fellow
Erasmus University
Rotterdam, The Netherlands
1996-2009 Grant Funding: $299,000
Summary
The Dutch-Belgian Fragile X Consortium led by Dr. Oostra created the first Fragile X mouse model - the FMR1 knockout mouse - and went on to perform many critical studies using this model. With $299,000 in funding from FRAXA Research Foundation from 1996-2009, Dr. Ben Oostra and his team at Erasmus University completed and published multiple studies related to Fragile X syndrome.
The Results
The overall objective for these projects was to understand the function of the FMR1 gene in the brain and the deleterious effect on brain functioning if the FMR1 gene is not working properly.
The original FMR1 knockout mouse model was published in the journal Cell in July 1994 by The Dutch-Belgian Fragile X Consortium.
The FMR1 knockout mouse and related models are now widely available, and a large number of studies have been published using these models. Without them, the Fragile X research field could not have progressed as it has in recent decades.
