Ultrastructural analysis of the functional domains in FMRP using primary hippocampal mouse neurons.

Josien Levenga 1 Ronald Buijsen 1 Maria Rifé 1 Hervé Moine 2 David Nelson 3 Ben Oostra 1 Rob Willemsen 1 Femke De Vrij 1
1 CBG Department of Clinical Genetics
2 IGBMC - Institut de génétique et biologie moléculaire et cellulaire
3 Department of Molecular and Human Genetics
Abstract : Fragile X syndrome is caused by lack of the protein FMRP. FMRP mediates mRNA binding, dendritic mRNA transport and translational control at spines. We examined the role of functional domains of FMRP in neuronal RNA-granule formation and dendritic transport using different FMRP variants, including the mutant FMRP_I304N and the splice-variant FMRP_Iso12. Both variants are absent from dendritic RNA-granules in Fmr1 knockout neurons. Co-transfection experiments showed that wild-type FMRP recruits both FMRP variants into dendritic RNA-granules. Co-transfection of FXR2, an FMRP homologue, also resulted in redistribution of both variants into dendritic RNA-granules. Furthermore, the capacity of the variants to transport their mRNAs and the mRNA localization of an FMR1 construct containing silent point-mutations affecting only the G-quartet-structure were investigated. In conclusion, we show that wild-type FMRP and FXR2P are able to recruit FMRP variants into RNA-granules and that the G-quartet-structure in FMR1 mRNA is not essential for its incorporation in RNA-granules.
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Neurobiology of Disease, Elsevier, 2009, 35 (2), pp.241-50. 〈10.1016/j.nbd.2009.05.004〉
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http://www.hal.inserm.fr/inserm-00420367
Contributeur : Maité Peney <>
Soumis le : lundi 28 septembre 2009 - 17:30:55
Dernière modification le : jeudi 11 janvier 2018 - 06:17:42

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Josien Levenga, Ronald Buijsen, Maria Rifé, Hervé Moine, David Nelson, et al.. Ultrastructural analysis of the functional domains in FMRP using primary hippocampal mouse neurons.. Neurobiology of Disease, Elsevier, 2009, 35 (2), pp.241-50. 〈10.1016/j.nbd.2009.05.004〉. 〈inserm-00420367〉

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