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Functional Analysis of Genes Implicated in Down Syndrome: 2. Laterality and Corpus Callosum Size in Mice Transpolygenic for Down Syndrome Chromosomal Region −1 (DCR-1)

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The association between atypical laterality and mental retardation has been reported several times, particularly in Down syndrome (DS). We investigated common genetic correlates of these components of the syndrome, examining direction (number of right paw entries in the Collins test) and degree (absolute difference between the number of right paw entries and the number of left paw entries) in mice that had incorporated extra-contiguous HSA21 fragments covering DCR-1 (Down Chromosomal Region-1). As corpus callosum size is substantially reduced in DS, and as the structure has been suspected of playing a role in atypical laterality, we also measured the corpus callosum in these mice. Extra copies of two regions (F7 and E6) have been associated with an atypical degree of laterality (strongly reduced degree). Extra copies of E8, G6 and E6 are also linked to the reduced size of the corpus callosum, indicating that the abnormal number of fibers linking the two hemispheres is not associated with atypical laterality in DS. Together, these results indicate that some of the genes involved in atypical laterality and in the reduced size of the corpus callosum in DS are present on DCR-1. An extra copy of F7 and, to a lesser extent, an extra copy of E6, are also associated with cognitive impairment. These results support the hypothesis of common genetic correlates in atypical laterality and mental retardation in DS.

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Author notes

  1. Zoë Bichler

    Present address: Molekulare Neurobiochemie, Ruhr-University Bochum, Germany

  2. Pierre L. Roubertoux

    Present address: Génomique Fonctionnelle, Pathologies, Comportements, P3M, UMR CNRS Marseille, France

Authors and Affiliations

  1. Génétique, Neurogénétique, Comportement, CNRS, France

    Pierre L. Roubertoux, Zoë Bichler, Walter Pinoteau, Danièle Migliore-Samour & Michèle Carlier

  2. Génomique Fonctionnelle, Pathologies, Comportements, P3M, UMR CNRS Marseille, France

    Zohra Seregaza & Marc Jamon

  3. Department of Molecular and Medical Pharmacology, UCLA School of Medicine, 23-120 CHS, Box 951735, Los Angeles, CA, 90095-1735, USA

    Sylvia Fortes & Desmond J. Smith

  4. Genome Sciences Department, Lawrence Berkeley National Laboratory, 1 Cyclotron Road, MS 84-171, Berkeley, CA, 94720, USA

    Edward Rubin

  5. Université de Provence and Institut Universitaire de France, France

    Michèle Carlier

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  1. Pierre L. Roubertoux
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Roubertoux, P.L., Bichler, Z., Pinoteau, W. et al. Functional Analysis of Genes Implicated in Down Syndrome: 2. Laterality and Corpus Callosum Size in Mice Transpolygenic for Down Syndrome Chromosomal Region −1 (DCR-1). Behav Genet 35, 333–341 (2005). https://doi.org/10.1007/s10519-005-3225-0

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