title: Overexpression of mSim2 gene in the zona limitans of the diencephalon of segmental trisomy 16 Ts1Cje fetuses, a mouse model for trisomy 21: a novel whole-mount based RNA hybridization study.
abstract: Trisomy 21 (Down syndrome) is the most common chromosomal abnormality associated with mental retardation in humans. Sim2, a human homologue of Drosophila sim gene, which acts as a master regulator of the early development of the fly central nervous system midline, is located on chromosome 21, in the Down syndrome critical region, and might therefore be involved in the pathogenesis of some of the morphological features and brain anomalies observed in Down syndrome. We report here the detailed expression pattern of murine mSim2 gene in Ts1Cje mice fetuses, a segmental trisomy 16 mouse model for trisomy 21, and its overexpression in the zona limitans of the diencephalon using a new quantitative method based on the whole-mount RNA hybridization technique.
authors.affiliation: CNRS UMR 8602, Faculté de Médecine Necker-Enfants Malades, 156 Rue de Vaugirard, 75730 Cedex 15, Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: Vialard
authors.forename: K
authors.initials: K
authors.lastname: Toyama
authors.forename: S
authors.initials: S
authors.lastname: Vernoux
authors.forename: E J
authors.initials: EJ
authors.lastname: Carlson
authors.forename: C J
authors.initials: CJ
authors.lastname: Epstein
authors.forename: P M
authors.initials: PM
authors.lastname: Sinet
authors.forename: Z
authors.initials: Z
authors.lastname: Rahmani
mesh_terms: D000818:Animals; D051792:Basic Helix-Loop-Helix Proteins; D004268:DNA-Binding Proteins / Q000235:genetics*; D004027:Diencephalon / Q000196:embryology / Q000502:physiology*; D004195:Disease Models, Animal; D004314:Down Syndrome / Q000235:genetics*; D029721:Drosophila Proteins; D005314:Embryonic and Fetal Development / Q000502:physiology; D005260:Female; D018507:Gene Expression Regulation, Developmental / Q000502:physiology*; D006801:Humans; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D009687:Nuclear Proteins / Q000235:genetics*; D009693:Nucleic Acid Hybridization; D011401:Promoter Regions, Genetic / Q000502:physiology
chemical_list: D051792:Basic Helix-Loop-Helix Proteins; D004268:DNA-Binding Proteins; D029721:Drosophila Proteins; D009687:Nuclear Proteins; C417032:SIM2 protein, human; C089449:sim protein, Drosophila