title: Gene expression profiles in the cerebellum  of transgenic mice over expressing the  human FMR1 gene with CGG repeats  in  the normal range.
abstract: Modifications in the GABA pathway are considered to be  responsible for motor alterations in animal models for fragile X-associated  tremor ataxia syndrome. We analyzed the expression profile in the cerebellum in  a transgenic mouse model that over expresses the human FMR1 gene with  CGG repeats in the normal range. We used the "GeneChip Mouse Gene 1.0 ST Array"  from Affymetrix analyzing 28,853 well-described and -characterized genes. Based  on data from the comparative analysis of the expression profile, we detected a  significant gradient with a P value <0.1 and changes in expression equal to  or greater than 1.5 times compared to the control mouse genes. There were  significant changes in the expression of 104 genes, among which 72% had  decreased and 28% had increased expression. With the exception of GabarapL2,  no changes in expression of genes from the GABA pathway were observed, which  may explain the absence of an altered motor phenotype in these mice. These  results further support the view that toxic effects in fragile X-associated  tremor ataxia syndrome are due to expansion of CGG repeats rather than  increased mRNA levels, since in the transgenic mice the FMR1 mRNA levels  were increased 20-100 times compared with those of control littermates.
authors.affiliation: Department of Medical Biochemistry and Molecular Biology, University Hospital Virgen Macarena, University of Seville, Spain.
authors.forename: J J
authors.initials: JJ
authors.lastname: Fernández
authors.forename: R
authors.initials: R
authors.lastname: Martínez
authors.forename: E
authors.initials: E
authors.lastname: Andújar
authors.forename: M
authors.initials: M
authors.lastname: Pérez-Alegre
authors.forename: A
authors.initials: A
authors.lastname: Costa
authors.forename: V
authors.initials: V
authors.lastname: Bonilla-Henao
authors.forename: F
authors.initials: F
authors.lastname: Sobrino
authors.forename: C Ó
authors.initials: CÓ
authors.lastname: Pintado
authors.forename: E
authors.initials: E
authors.lastname: Pintado
mesh_terms: D000818:Animals; D002531:Cerebellum / Q000166:cytology*; D004195:Disease Models, Animal; D051860:Fragile X Messenger Ribonucleoprotein 1 / Q000096:biosynthesis* / Q000235:genetics*; D005600:Fragile X Syndrome / Q000235:genetics; D020869:Gene Expression Profiling; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D020411:Oligonucleotide Array Sequence Analysis; D012333:RNA, Messenger / Q000096:biosynthesis; D015398:Signal Transduction / Q000235:genetics; D059467:Transcriptome; D019680:Trinucleotide Repeat Expansion / Q000235:genetics*
chemical_list: D051860:Fragile X Messenger Ribonucleoprotein 1; D012333:RNA, Messenger; C497683:FMR1 protein, human