http://www.cnr.it/ontology/cnr/individuo/prodotto/ID171580

Gene expression profiles in a transgenic animal model of fragile x syndrome (Articolo in rivista)

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Gene expression profiles in a transgenic animal model of fragile x syndrome (Articolo in rivista) (literal)

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DAgata V., Warren S. T., Zhao W., Torre E. R., Alkon D. L., and Cavallaro S. (2002)
Gene expression profiles in a transgenic animal model of fragile x syndrome
in Neurobiology of disease
(literal)

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DAgata V., Warren S. T., Zhao W., Torre E. R., Alkon D. L., and Cavallaro S. (literal)

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The observation that FMRP is an RNA binding protein and may be implicated in RNA metabolism suggests that other genes, whose products may vary in the absence of FMRP, could play a significant role in the cognitive deficits associated with fragile X syndrome. The generation of FMR1 knockout (KO) mice by homologous recombination (The Dutch-Belgian Fragile X Consortium, 1994) provides an animal model to test this hypothesis. Indeed, FMR1 knockout mice show macroorchidism, hyperactivity and behavioral deficits, abnormalities that mimic the human syndrome. To gain greater insight into the molecular mechanisms leading to mental retardation in fragile X syndrome, we have used the unprecedented experimental opportunities that the genome sequences and the development of cDNA array technology now provide to perform genome-wide expression analysis in brains of wild-type and FMR1 knockout mice. (literal)

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1 Institute of Neurological Sciences, CNR, Mangone (Italy) 2 Howard Hughes Medical Institute and Department of Biochemistry, Pediatrics and Genetics, Emory University School of Medicine, Atlanta, Georgia,30322. (literal)

Titolo

Gene expression profiles in a transgenic animal model of fragile x syndrome (literal)

Abstract

Fragile X syndrome is the most common inherited form of mental retardation. Although this syndrome originates from the absence of the RNA-binding protein FMRP, the molecular mechanisms underlying the cognitive deficits are unknown. The expression pattern of 6789 genes was studied in the brains of wild-type and FMR1 knockout mice, a fragile X syndrome animal model that has been associated with cognitive deficits. Differential expression of more than two-fold was observed for the brain mRNA levels of 73 genes. Differential expression of nine of these genes was confirmed by real-time quantitative reverse transcription-polymerase chain reaction. and by in situ hybridization. In addition to corroborating the microarray data, the in situ hybridization analysis showed distinct spatial distribution patterns of microtubule-associated protein 2 and amyloid beta precursor protein. A number of differentially expressed genes associated with the fragile X syndrome phenotype have been previously involved in other memory or cognitive disorders. (literal)

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