Journal compilation # 2007 Blackwell Publishing Ltd Review Developmental mechanisms and experimental models to understand forebrain malformative diseases

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 verte-brate 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 themajor stages in forebrain morphogenesis and region-alization, with special emphasis on developmental molecular mechanisms derailing telencephalic develop-ment with subsequent damage to cortical function. Because animal models, mainly mouse, have been fun-damental 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. Althoughmost of the genes involved in the regulation of basic developmental pro-cesses are conserved among vertebrates, the extrapola-tion of mouse data to corresponding gene expression and function in humans needs careful individual analysis in each functional system