The Structure of the Cerebral Cortex in Down's Syndrome – A quantitative analysis –

Colon, E. J.

doi:10.1055/s-0028-1091775

Cited by 55 publications

(25 citation statements)

References 3 publications

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“…There is also evidence for abnormalities of neuronal differentiation and abnormal migration in fetal and infant brain [21]. Neuronal loss is prominent from birth onwards [11,14,82] but a consistent picture of the "wiring'' of the brain in DS has not yet emerged.…”

Section: Introductionmentioning

confidence: 99%

The brain in Down syndrome (TRISOMY 21)

Lubec

Engidawork

2002

Journal of Neurology

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Down syndrome (DS) is the most common genetic birth defect associated with mental retardation. The mechanism(s) underlying the neuropathology of DS is not completely understood. Different hypotheses have been advanced to explain this mystery, including the gene dosage effect, the amplified developmental instability, and the molecular misreading concept. Overexpression of genes residing in chromosome 21 has been assumed to be a central point in the neuropathology of DS, although reports disagreeing with this notion have also been published. In addition, an accumulating body of evidence indicates that genes located on other chromosomes are also involved in the process. DS thus appears to be a disease process involving numerous gene products and this interaction and interplay in the final analysis determines the outcome of the disease. In this regard, transcription factors, reactive oxygen species and apoptosis related proteins are viewed as potential candidates that play a significant role in the disease process. Therapeutic modalities that target these factors including antioxidants and caspase inhibitors might have some benefit in alleviating the symptoms of DS.

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Section: Introductionmentioning

confidence: 99%

The brain in Down syndrome (TRISOMY 21)

Lubec

Engidawork

2002

Journal of Neurology

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“…Histopathological observations of prenatal DS brains revealed that fewer neurons are present in specific layers of the cortex when compared to euploid brains [Davidoff, 1928;Benda, 1947;Crome and Stern, 1967;Colon, 1972;Ross et al, 1984;Wisniewski et al, 1984;Becker et al, 1991;Golden and Hyman, 1994], and this lack of cortical neurons occurs at a specific developmental time ( 1 23 weeks) [Ross et al, 1984;Golden and Hyman, 1994]. Trisomy 21 hNPCs in culture generate fewer neurons than euploid controls and can therefore be used to define the mechanism of reduced cortical neurogenesis in trisomy 21 [Bahn et al, 2002;Bhattacharyya and Svendsen, 2003].…”

Section: Temporal Changes In Neurogenesis From Trisomy 21 Hnpcsmentioning

confidence: 99%

“…Overall, DS brains weigh less, and the cerebral cortices have simpler convolutions than agematched euploid brains [Becker et al, 1991;Colon, 1972;Schmidt-Sidor et al, 1990;Wisniewski, 1990]. A dramatic reduction in the number of neurons in the developing cerebral cortex of DS patients has been consistently reported dating back to the 1920s [Becker et al, 1991;Benda, 1947;Colon, 1972;Crome and Stern, 1967;Davidoff, 1928;Golden and Hyman, 1994;Ross et al, 1984;Wisniewski et al, 1984]. It is important to note that the neuron reductions are area, cell type and age specific: the missing neurons are primarily small, granular, presumably GABAergic neurons in layer II and layer IV of the cortex [Ross et al, 1984].…”

Section: Introductionmentioning

confidence: 99%

A Critical Period in Cortical Interneuron Neurogenesis in Down Syndrome Revealed by Human Neural Progenitor Cells

et al. 2009

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Down syndrome (DS) is a developmental disorder whose mental impairment is due to defective cortical development. Human neural progenitor cells (hNPCs) derived from fetal DS cortex initially produce normal numbers of neurons, but generate fewer neurons with time in culture, similar to the pattern of neurogenesis that occurs in DS in vivo. Microarray analysis of DS hNPCs at this critical time reveals gene changes indicative of defects in interneuron progenitor development. In addition, dysregulated expression of many genes involved in neural progenitor cell biology points to changes in the progenitor population and subsequent reduction in interneuron neurogenesis. Delineation of a critical period in interneuron development in DS provides a foundation for investigation of the basis of reduced neurogenesis in DS and defines a time when these progenitor cells may be amenable to therapeutic treatment.

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“…Similarly, the reduced brain size found in Down syndrome (DS) [Colon, 1972;Schmidt-Sidor et al, 1990;Wisniewski, 1990;Wisniewski et al, 1993] and microcephaly vera [Mochida and Walsh, 2001] may be due to altered cell divisions that occur during Stages 1 and 2. These changes can be as subtle as a slight increase in cell cycle duration, such as that found in DS [Schneider and Epstein, 1972;Bernert et al, 1996] and DS mouse models [Haydar et al, 2000a], which leads to the production of fewer VZ founder cells and a delay in the radial expansion of the cerebral wall during neurogenesis.…”

Section: Expansion and Commitment Of Neural Precursorsmentioning

confidence: 95%

Advanced microscopic imaging methods to investigate cortical development and the etiology of mental retardation

Haydar

2005

Ment. Retard. Dev. Disabil. Res. Rev.

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Studies on human patients and animal models of disease have shown that disruptions in prenatal and early postnatal brain development are a root cause of mental retardation. Since proper brain development is achieved by a strict spatiotemporal control of neurogenesis, cell migration, and patterning of synapses, abnormalities in one or more of these events during prenatal development can lead to cognitive dysfunction after birth. Many of underlying causes of mental retardation must therefore be studied in developing brains. To aid in this research, live imaging using laser scanning microscopy (LSM) has recently allowed neuroscientists to delve deeply into the complex three-dimensional environment of the living brain to record dynamic cellular events over time. This review will highlight recent examples of how LSM is being applied to elucidate both normal and abnormal cortical development.

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The Structure of the Cerebral Cortex in Down's Syndrome – A quantitative analysis –

Cited by 55 publications

References 3 publications

The brain in Down syndrome (TRISOMY 21)

The brain in Down syndrome (TRISOMY 21)

A Critical Period in Cortical Interneuron Neurogenesis in Down Syndrome Revealed by Human Neural Progenitor Cells

Advanced microscopic imaging methods to investigate cortical development and the etiology of mental retardation

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