Consequences of Trisomy 16 for Mouse Brain Development: Corticogenesis in a Model of Down Syndrome

We have studied abnormalities in the tangential and radial expansion of the cerebral cortex during fetal development in the trisomy 16 (Ts16) mouse, a model for human trisomy 21 (Down syndrome). Slowed tangential expansion of the neuroepithelium in Ts16 resulted in a reduction of final telencephalic size and is predicted to decrease the number of radial cortical units in the mature brain. In addition, radial growth of the Ts16 cortex was delayed at the time of peak cortical neurogenesis in normal mice, but by embryonic day 18 the cortex reached normal thickness. Because mouse chromosome 16 shares many genes with human chromosome 21, abnormalities in Ts16 brain development may parallel abnormalities in trisomy 21.

[1]  K. Wisniewski,et al.  Down syndrome children often have brain with maturation delay, retardation of growth, and cortical dysgenesis. , 2005, American journal of medical genetics. Supplement.

[2]  A. Blaschke,et al.  Widespread programmed cell death in proliferative and postmitotic regions of the fetal cerebral cortex. , 1996, Development.

[3]  C. Frassoni,et al.  In situ labeling of apoptotic cell death in the cerebral cortex and thalamus of rats during development , 1995, The Journal of comparative neurology.

[4]  P. Rakic.  Radial versus tangential migration of neuronal clones in the developing cerebral cortex. , 1995, Proceedings of the National Academy of Sciences of the United States of America.

[5]  A. Kriegstein,et al.  GABA and glutamate depolarize cortical progenitor cells and inhibit DNA synthesis , 1995, Neuron.

[6]  P. Yarowsky,et al.  Glutamate as a hippocampal neuron survival factor: an inherited defect in the trisomy 16 mouse. , 1995, Proceedings of the National Academy of Sciences of the United States of America.

[7]  V. Caviness,et al.  Numbers, time and neocortical neuronogenesis: a general developmental and evolutionary model , 1995, Trends in Neurosciences.

[8]  P. Rakic,et al.  Radial and horizontal deployment of clonally related cells in the primate neocortex: Relationship to distinct mitotic lineages , 1995, Neuron.

[9]  B T Hyman,et al.  Development of the Superior Temporal Neocortex Is Anomalous in Trisomy 21 , 1994, Journal of neuropathology and experimental neurology.

[10]  A L Pearlman,et al.  Thalamocortical axons extend along a chondroitin sulfate proteoglycan- enriched pathway coincident with the neocortical subplate and distinct from the efferent path , 1994, The Journal of neuroscience : the official journal of the Society for Neuroscience.

[11]  C. Shatz,et al.  Subplate pioneers and the formation of descending connections from cerebral cortex , 1994, The Journal of neuroscience : the official journal of the Society for Neuroscience.

[12]  V. Caviness,et al.  Mode of cell proliferation in the developing mouse neocortex. , 1994, Proceedings of the National Academy of Sciences of the United States of America.

[13]  S. Rapoport,et al.  Cultured hippocampal neurons from trisomy 16 mouse, a model for Down's syndrome, have an abnormal action potential due to a reduced inward sodium current , 1993, Brain Research.

[14]  D. O'Leary,et al.  Growth and targeting of subplate axons and establishment of major cortical pathways [published erratum appears in J Neurosci 1993 Mar;13(3):following table of contents] , 1992, The Journal of neuroscience : the official journal of the Society for Neuroscience.

[15]  D. O'Leary,et al.  Potential of visual cortex to develop an array of functional units unique to somatosensory cortex , 1991, Science.

[16]  L. Becker.  Synaptic Dysgenesis , 1991, Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques.

[17]  I. Ferrer,et al.  Naturally occurring cell death in the cerebral cortex of the rat and removal of dead cells by transitory phagocytes , 1990, Neuroscience.

[18]  R S Erzurumlu,et al.  Thalamic axons confer a blueprint of the sensory periphery onto the developing rat somatosensory cortex. , 1990, Brain research. Developmental brain research.

[19]  K. Wisniewski,et al.  Brain growth in Down syndrome subjects 15 to 22 weeks of gestational age and birth to 60 months. , 1990, Clinical neuropathology.

[20]  J. Coyle,et al.  Neurogenesis of the basal forebrain in euploid and trisomy 16 mice: An animal model for developmental disorders in down syndrome , 1989, Neuroscience.

[21]  R. Balázs,et al.  Selective retardation of the development of the basal forebrain cholinergic and pontine catecholaminergic nuclei in the brain of trisomy 16 mouse, an animal model of Down's syndrome. , 1989, Brain research. Developmental brain research.

[22]  P. Rakic.  Specification of cerebral cortical areas. , 1988, Science.

[23]  S. Mcconnell.  Development and decision-making in the mammalian cerebral cortex , 1988, Brain Research Reviews.

[24]  J. Sanes,et al.  Use of a recombinant retrovirus to study post‐implantation cell lineage in mouse embryos. , 1986, The EMBO journal.

[25]  J. Gearhart,et al.  Genetic basis for a mouse model of down syndrome , 1986, Brain Research Bulletin.

[26]  M. Oster-Granite,et al.  Autosomal aneuploidy in mice: Generation and developmental consequences , 1986, Brain Research Bulletin.

[27]  H. Wiśniewski,et al.  Evidence of arrest of neurogenesis and synaptogenesis in brains of patients with Down's syndrome. , 1984, The New England journal of medicine.

[28]  A. Galaburda,et al.  Down's syndrome , 1984, Neurology.

[29]  P. Rakić,et al.  Early developmental events: cell lineages, acquisition of neuronal positions, and areal and laminar development. , 1982, Neurosciences Research Program bulletin.

[30]  I. Smart,et al.  Growth patterns in the lateral wall of the mouse telencephalon: I. Autoradiographic studies of the histogenesis of the isocortex and adjacent areas. , 1982, Journal of anatomy.

[31]  P. Layde,et al.  Down's Syndrome: Recent Trends in the United States , 1981 .

[32]  D. Frost,et al.  Tangential organization of thalamic projections to the neocortex in the mouse , 1980, The Journal of comparative neurology.

[33]  D. Segal,et al.  Studies on Down's syndrome in tissue culture. I. Growth rates protein contents of fibroblast cultures , 1974, Journal of cellular physiology.

[34]  E. Colon.  The Structure of the Cerebral Cortex in Down's Syndrome – A quantitative analysis – , 1972 .

[35]  P. Rakić.  Mode of cell migration to the superficial layers of fetal monkey neocortex , 1972, The Journal of comparative neurology.

[36]  M. Jacobson,et al.  Embryonic vertebrate central nervous system: Revised terminology , 1970 .

[37]  V. Mountcastle.  Modality and topographic properties of single neurons of cat's somatic sensory cortex. , 1957, Journal of neurophysiology.

[38]  L. Davidoff.  THE BRAIN IN MONGOLIAN IDIOCY: A REPORT OF TEN CASES , 1928 .

[39]  Oster-Granite Ml,et al.  The neuropathology of the trisomy 16 mouse. , 1994 .

[40]  M. Oster-Granite,et al.  The neuropathology of the trisomy 16 mouse. , 1994, Critical Reviews in Neurobiology.

[41]  J. Richtsmeier,et al.  Morphogenesis of the brain and craniofacial complex in trisomy 16 mice. , 1991, Progress in clinical and biological research.

[42]  I. Kola.  Molecular genetics of Down syndrome. , 1989, Reproduction, fertility, and development.

[43]  M. Marín‐Padilla,et al.  Early Ontogenesis of the Human Cerebral Cortex , 1988 .

[44]  C. Cepko,et al.  Lineage analysis in the vertebrate nervous system by retrovirus-mediated gene transfer. , 1987, Proceedings of the National Academy of Sciences of the United States of America.

[45]  P. Gerald,et al.  Trisomy 21 (Down syndrome) : research perspectives , 1981 .

[46]  A. Gropp,et al.  Trisomy in the fetal backcross progeny of male and female metacentric heterozygotes of the mouse. i. , 1974, Cytogenetics and cell genetics.

[47]  Embryonic vertebrate central nervous system: revised terminology. The Boulder Committee. , 1970, The Anatomical record.