Gene Review:
Fmr1 - fragile X mental retardation syndrome 1
Mus musculus
Synonyms:
FMRP, Fmr-1, Fragile X mental retardation protein 1 homolog, Protein FMR-1, mFmr1p
- Macroorchidism in FMR1 knockout mice is caused by increased Sertoli cell proliferation during testicular development. Slegtenhorst-Eegdeman, K.E., de Rooij, D.G., Verhoef-Post, M., van de Kant, H.J., Bakker, C.E., Oostra, B.A., Grootegoed, J.A., Themmen, A.P. Endocrinology (1998)
- Prolonged epileptiform discharges induced by altered group I metabotropic glutamate receptor-mediated synaptic responses in hippocampal slices of a fragile X mouse model. Chuang, S.C., Zhao, W., Bauchwitz, R., Yan, Q., Bianchi, R., Wong, R.K. J. Neurosci. (2005)
- Object recognition impairment in Fmr1 knockout mice is reversed by amphetamine: involvement of dopamine in the medial prefrontal cortex. Ventura, R., Pascucci, T., Catania, M.V., Musumeci, S.A., Puglisi-Allegra, S. Behavioural pharmacology. (2004)
- Fragile X mental retardation protein shifts between polyribosomes and stress granules after neuronal injury by arsenite stress or in vivo hippocampal electrode insertion. Kim, S.H., Dong, W.K., Weiler, I.J., Greenough, W.T. J. Neurosci. (2006)
- Deletion of FMR1 in Purkinje cells enhances parallel fiber LTD, enlarges spines, and attenuates cerebellar eyelid conditioning in Fragile X syndrome. Koekkoek, S.K., Yamaguchi, K., Milojkovic, B.A., Dortland, B.R., Ruigrok, T.J., Maex, R., De Graaf, W., Smit, A.E., VanderWerf, F., Bakker, C.E., Willemsen, R., Ikeda, T., Kakizawa, S., Onodera, K., Nelson, D.L., Mientjes, E., Joosten, M., De Schutter, E., Oostra, B.A., Ito, M., De Zeeuw, C.I. Neuron (2005)
- Exaggerated behavioral phenotypes in Fmr1/Fxr2 double knockout mice reveal a functional genetic interaction between Fragile X-related proteins. Spencer, C.M., Serysheva, E., Yuva-Paylor, L.A., Oostra, B.A., Nelson, D.L., Paylor, R. Hum. Mol. Genet. (2006)
- Mildly impaired water maze performance in male Fmr1 knockout mice. D'Hooge, R., Nagels, G., Franck, F., Bakker, C.E., Reyniers, E., Storm, K., Kooy, R.F., Oostra, B.A., Willems, P.J., De Deyn, P.P. Neuroscience (1997)
- Spatial learning, contextual fear conditioning and conditioned emotional response in Fmr1 knockout mice. Van Dam, D., D'Hooge, R., Hauben, E., Reyniers, E., Gantois, I., Bakker, C.E., Oostra, B.A., Kooy, R.F., De Deyn, P.P. Behav. Brain Res. (2000)
- Social behavior deficits in the Fmr1 mutant mouse. Mineur, Y.S., Huynh, L.X., Crusio, W.E. Behav. Brain Res. (2006)
- Altered anxiety-related and social behaviors in the Fmr1 knockout mouse model of fragile X syndrome. Spencer, C.M., Alekseyenko, O., Serysheva, E., Yuva-Paylor, L.A., Paylor, R. Genes Brain Behav. (2005)
- The fragile X syndrome protein FMRP associates with BC1 RNA and regulates the translation of specific mRNAs at synapses. Zalfa, F., Giorgi, M., Primerano, B., Moro, A., Di Penta, A., Reis, S., Oostra, B., Bagni, C. Cell (2003)
- Microarray identification of FMRP-associated brain mRNAs and altered mRNA translational profiles in fragile X syndrome. Brown, V., Jin, P., Ceman, S., Darnell, J.C., O'Donnell, W.T., Tenenbaum, S.A., Jin, X., Feng, Y., Wilkinson, K.D., Keene, J.D., Darnell, R.B., Warren, S.T. Cell (2001)
- Enhanced Fmr-1 expression in testis. Bächner, D., Stéinbach, P., Wöhrle, D., Just, W., Vogel, W., Hameister, H., Manca, A., Poustka, A. Nat. Genet. (1993)
- Kissing complex RNAs mediate interaction between the Fragile-X mental retardation protein KH2 domain and brain polyribosomes. Darnell, J.C., Fraser, C.E., Mostovetsky, O., Stefani, G., Jones, T.A., Eddy, S.R., Darnell, R.B. Genes Dev. (2005)
- The generation of a conditional Fmr1 knock out mouse model to study Fmrp function in vivo. Mientjes, E.J., Nieuwenhuizen, I., Kirkpatrick, L., Zu, T., Hoogeveen-Westerveld, M., Severijnen, L., Rifé, M., Willemsen, R., Nelson, D.L., Oostra, B.A. Neurobiol. Dis. (2006)
- Synaptic regulation of protein synthesis and the fragile X protein. Greenough, W.T., Klintsova, A.Y., Irwin, S.A., Galvez, R., Bates, K.E., Weiler, I.J. Proc. Natl. Acad. Sci. U.S.A. (2001)
- Isolation and characterization of brain Y-box protein: developmentally regulated expression, polyribosomal association and dendritic localization. Funakoshi, T., Kobayashi, S., Ohashi, S., Sato, T.A., Anzai, K. Brain Res. Mol. Brain Res. (2003)
- Early postnatal plasticity in neocortex of FMR1 knockout mice. Desai, N.S., Casimiro, T.M., Gruber, S.M., Vanderklish, P.W. J. Neurophysiol. (2006)
- A null mutation for Fmr1 in female mice: effects on regional cerebral metabolic rate for glucose and relationship to behavior. Qin, M., Kang, J., Smith, C.B. Neuroscience (2005)
- Mean corpuscular hemoglobin is not increased in Fmr1 knockout mice. Reyniers, E., Van Bockstaele, D.R., De Boulle, K., Kooy, R.F., Bakker, C.E., Oostra, B.A., Willems, P.J. Hum. Genet. (1996)
- Suppression of two major Fragile X Syndrome mouse model phenotypes by the mGluR5 antagonist MPEP. Yan, Q.J., Rammal, M., Tranfaglia, M., Bauchwitz, R.P. Neuropharmacology (2005)
- The fragile X mental retardation protein and group I metabotropic glutamate receptors regulate levels of mRNA granules in brain. Aschrafi, A., Cunningham, B.A., Edelman, G.M., Vanderklish, P.W. Proc. Natl. Acad. Sci. U.S.A. (2005)
- Long-term potentiation in the hippocampus of fragile X knockout mice. Godfraind, J.M., Reyniers, E., De Boulle, K., D'Hooge, R., De Deyn, P.P., Bakker, C.E., Oostra, B.A., Kooy, R.F., Willems, P.J. Am. J. Med. Genet. (1996)
- A reduced number of metabotropic glutamate subtype 5 receptors are associated with constitutive homer proteins in a mouse model of fragile X syndrome. Giuffrida, R., Musumeci, S., D'Antoni, S., Bonaccorso, C.M., Giuffrida-Stella, A.M., Oostra, B.A., Catania, M.V. J. Neurosci. (2005)
- S6K1 phosphorylates and regulates fragile X mental retardation protein (FMRP) with the neuronal protein synthesis-dependent mammalian target of rapamycin (mTOR) signaling cascade. Narayanan, U., Nalavadi, V., Nakamoto, M., Thomas, G., Ceman, S., Bassell, G.J., Warren, S.T. J. Biol. Chem. (2008)
- Developmentally-programmed FMRP expression in oligodendrocytes: a potential role of FMRP in regulating translation in oligodendroglia progenitors. Wang, H., Ku, L., Osterhout, D.J., Li, W., Ahmadian, A., Liang, Z., Feng, Y. Hum. Mol. Genet. (2004)
- Fxr1 knockout mice show a striated muscle phenotype: implications for Fxr1p function in vivo. Mientjes, E.J., Willemsen, R., Kirkpatrick, L.L., Nieuwenhuizen, I.M., Hoogeveen-Westerveld, M., Verweij, M., Reis, S., Bardoni, B., Hoogeveen, A.T., Oostra, B.A., Nelson, D.L. Hum. Mol. Genet. (2004)
- The fragile X mental retardation protein is required for type-I metabotropic glutamate receptor-dependent translation of PSD-95. Todd, P.K., Mack, K.J., Malter, J.S. Proc. Natl. Acad. Sci. U.S.A. (2003)
- Rgs4 mRNA expression is decreased in the brain of Fmr1 knockout mouse. Tervonen, T., Akerman, K., Oostra, B.A., Castrén, M. Brain Res. Mol. Brain Res. (2005)
- Mapping of FMR1, the gene implicated in fragile X-linked mental retardation, on the mouse X chromosome. Laval, S.H., Blair, H.J., Hirst, M.C., Davies, K.E., Boyd, Y. Genomics (1992)
- Fragile X mental retardation protein FMRP and the RNA export factor NXF2 associate with and destabilize Nxf1 mRNA in neuronal cells. Zhang, M., Wang, Q., Huang, Y. Proc. Natl. Acad. Sci. U.S.A. (2007)
- A gene-based genetic linkage and comparative map of the rat X chromosome. Millwood, I.Y., Bihoreau, M.T., Gauguier, D., Hyne, G., Levy, E.R., Kreutz, R., Lathrop, G.M., Monaco, A.P. Genomics (1997)
- Identification of mRNA/protein (mRNP) complexes containing Puralpha, mStaufen, fragile X protein, and myosin Va and their association with rough endoplasmic reticulum equipped with a kinesin motor. Ohashi, S., Koike, K., Omori, A., Ichinose, S., Ohara, S., Kobayashi, S., Sato, T.A., Anzai, K. J. Biol. Chem. (2002)
- Prospects of TAT-mediated protein therapy for fragile X syndrome. Reis, S.A., Willemsen, R., van Unen, L., Hoogeveen, A.T., Oostra, B.A. J. Mol. Histol. (2004)