title: Lamination of the cerebral cortex is disturbed in Gli3 mutant mice.
abstract: The layered organization of the cerebral cortex develops in an inside-out pattern, a process which is controlled by the secreted protein reelin. Here we report on cortical lamination in the Gli3 hypomorphic mouse mutant Xt(J)/Pdn which lacks the cortical hem, a major source of reelin(+) Cajal Retzius cells in the cerebral cortex. Unlike other previously described mouse mutants with hem defects, cortical lamination is disturbed in Xt(J)/Pdn animals. Surprisingly, these layering defects occur in the presence of reelin(+) cells which are probably derived from an expanded Dbx1(+) progenitor pool in the mutant. However, while these reelin(+) neurons and also Calretinin(+) cells are initially evenly distributed over the cortical surface they form clusters later during development suggesting a novel role for Gli3 in maintaining the proper arrangement of these cells in the marginal zone. Moreover, the radial glial network is disturbed in the regions of these clusters. In addition, the differentiation of subplate cells is affected which serve as a framework for developing a properly laminated cortex.
authors.affiliation: Institute for Animal Developmental and Molecular Biology, Heinrich-Heine-University, D-40225 Düsseldorf, Germany.
authors.forename: Melanie
authors.initials: M
authors.lastname: Friedrichs
authors.forename: Osmany
authors.initials: O
authors.lastname: Larralde
authors.forename: Thomas
authors.initials: T
authors.lastname: Skutella
authors.forename: Thomas
authors.initials: T
authors.lastname: Theil
mesh_terms: D000818:Animals; D060466:Axin Protein; D015415:Biomarkers / Q000378:metabolism; D015816:Cell Adhesion Molecules, Neuronal / Q000235:genetics / Q000378:metabolism; D002540:Cerebral Cortex / Q000033:anatomy & histology* / Q000196:embryology* / Q000378:metabolism; D003598:Cytoskeletal Proteins / Q000235:genetics / Q000378:metabolism; D004622:Embryo, Mammalian / Q000033:anatomy & histology / Q000502:physiology; D016326:Extracellular Matrix Proteins / Q000235:genetics / Q000378:metabolism; D006801:Humans; D017403:In Situ Hybridization; D051741:Kruppel-Like Transcription Factors / Q000235:genetics / Q000378:metabolism*; D051379:Mice; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000378:metabolism*; D009474:Neurons / Q000166:cytology / Q000378:metabolism; D000091042:Reelin Protein; D012697:Serine Endopeptidases / Q000235:genetics / Q000378:metabolism; D000074290:Zinc Finger Protein Gli3
chemical_list: D060466:Axin Protein; C111729:Axin2 protein, mouse; D015415:Biomarkers; D015816:Cell Adhesion Molecules, Neuronal; D003598:Cytoskeletal Proteins; D016326:Extracellular Matrix Proteins; C465571:Gli3 protein, mouse; D051741:Kruppel-Like Transcription Factors; D009419:Nerve Tissue Proteins; D000091042:Reelin Protein; D000074290:Zinc Finger Protein Gli3; C000717127:RELN protein, human; C000717128:Reln protein, mouse; D012697:Serine Endopeptidases