Present address: MRC Mammalian Genetics Unit, Harwell OX11 0RD, UK.
Localization of the fragile X mental retardation 2 (FMR2) protein in mammalian brain
Article first published online: 9 OCT 2008
European Journal of Neuroscience
Volume 12, Issue 1, pages 381–384, January 2000
How to Cite
Miller, W. J., Skinner, J. A., Foss, G. S. and Davies, K. E. (2000), Localization of the fragile X mental retardation 2 (FMR2) protein in mammalian brain. European Journal of Neuroscience, 12: 381–384. doi: 10.1046/j.1460-9568.2000.00921.x
- Issue published online: 9 OCT 2008
- Article first published online: 9 OCT 2008
- Received 23 June 1999, revised 14 October 1999, accepted 18 October 1999
- mental handicap;
The transcriptional silencing of the FMR2 gene has been implicated in FRAXE mental retardation. FRAXE individuals have been shown to exhibit learning deficits, including speech delay, reading and writing problems. FMR2 encodes a large protein of 1311 amino acids and is a member of a gene family encoding proline–serine-rich proteins that have properties of nuclear transcription factors. To characterize the expression of the fragile X mental retardation 2 (FMR2) protein, polyclonal antibodies were raised against two regions of the human FMR2 protein and used in immunofluorescence experiments on mouse brain cryosections. Our results demonstrate for the first time that the FMR2 protein is localized in neurons of the neocortex, Purkinje cells of the cerebellum and the granule cell layer of the hippocampus. FMR2 staining is shown to colocalize with the nuclear stain 4,6-diamidino-2-phenylindole (DAPI) confirming that FMR2 is a nuclear protein. The localization of FMR2 protein to the mammalian hippocampus and other brain structures involved with cognitive function is consistent with the learning deficits seen in FRAXE individuals.