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Publication : Developmental mechanisms and experimental models to understand forebrain malformative diseases.

First Author  Pombero A Year  2007
Journal  Genes Brain Behav Volume  6 Suppl 1
Pages  45-52 PubMed ID  17543039
Mgi Jnum  J:135094 Mgi Id  MGI:3790366
Doi  10.1111/j.1601-183X.2007.00322.x Citation  Pombero A, et al. (2007) Developmental mechanisms and experimental models to understand forebrain malformative diseases. Genes Brain Behav 6 Suppl 1:45-52
abstractText  The development of the central nervous system can be divided into a number of phases, each of which can be subject of genetic or epigenetic alterations that may originate particular developmental disorders. In recent years, much progress has been made in elucidating the molecular and cellular mechanisms by which the vertebrate forebrain develops. Therefore, our understanding of major developmental brain disorders such as cortical malformations and neuronal migration disorders has significantly increased. In this review, we will describe the major stages in forebrain morphogenesis and regionalization, with special emphasis on developmental molecular mechanisms derailing telencephalic development with subsequent damage to cortical function. Because animal models, mainly mouse, have been fundamental for this progress, we will also describe some characteristic mouse models that have been capital to explore these molecular mechanisms of malformative diseases of the human brain. Although most of the genes involved in the regulation of basic developmental processes are conserved among vertebrates, the extrapolation of mouse data to corresponding gene expression and function in humans needs careful individual analysis in each functional system.
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