title: Gene expression profiles in a transgenic animal model of fragile X syndrome.
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.
authors.affiliation: Blanchette Rockefeller Neurosciences Institute, West Virginia University, Rockville, MD 20850, USA.
authors.forename: Velia
authors.initials: V
authors.lastname: D'Agata
authors.forename: Stephen T
authors.initials: ST
authors.lastname: Warren
authors.forename: Weiqin
authors.initials: W
authors.lastname: Zhao
authors.forename: Enrique R
authors.initials: ER
authors.lastname: Torre
authors.forename: Daniel L
authors.initials: DL
authors.lastname: Alkon
authors.forename: Sebastiano
authors.initials: S
authors.lastname: Cavallaro
mesh_terms: D016564:Amyloid beta-Protein Precursor / Q000096:biosynthesis / Q000235:genetics; D000818:Animals; D001921:Brain / Q000378:metabolism; D004195:Disease Models, Animal; D005260:Female; D005600:Fragile X Syndrome / Q000235:genetics* / Q000378:metabolism; D020869:Gene Expression Profiling / Q000379:methods / Q000706:statistics & numerical data; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D008822:Mice, Transgenic; D008869:Microtubule-Associated Proteins / Q000096:biosynthesis / Q000235:genetics; D020411:Oligonucleotide Array Sequence Analysis / Q000379:methods / Q000706:statistics & numerical data
chemical_list: D016564:Amyloid beta-Protein Precursor; D008869:Microtubule-Associated Proteins