A Perspective: The Role of Disordered Genetic Control of Neurogenesis in the Pathogenesis of Migration Disorders

• Published in: Journal of neuropathology and experimental neurology Vol. 53; no. 2; pp. 105 - 117
• Main Author: Rorke, Lucy Balian
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD American Association of Neuropathologists, Inc 01.03.1994; Lippincott Williams & Wilkins
• Subjects: Animals; Biological and medical sciences; Brain Diseases - genetics; Brain Diseases - pathology; Caenorhabditis elegans; Caenorhabditis elegans - genetics; Cerebral Cortex - abnormalities; Cerebral Cortex - pathology; Drosophila; Drosophila - genetics; Humans; Malformations of the nervous system; Medical sciences; Neurology; Neurons - pathology; Human; Nervous system diseases; Cerebral cortex; Dysplasia; Genetic inheritance; Etiopathogenesis; Congenital disease; Pathology; Neuron; Lissencephaly; Malformation; Central nervous system disease; Development; Brain (vertebrata)
• ISSN: 0022-3069; 1554-6578
• DOI: 10.1097/00005072-199403000-00001

Morphological features of cerebral cortical dysplasias generally regarded under the umbrella term 'migration disorders' have been reviewed and critically analyzed against current knowledge of gene activity involved in neurogenesis. Although data are plentiful regarding the nature and role of these genes in lower forms, i.e. C. elegans and Drosophila, relatively few human homologs have been identified. Multiple genes are involved in various specific aspects of neurogenesis, i.e. neuroblast proliferation, PCD, migration, etc., and it is postulated that there are parallel gene actions in human neural development. A hypothetical construct of specific gene defects in human neurogenesis accounting for the morphological variations observed in the migration disorders is postulated.