Potential mechanisms of mutations that affect neuronal migration in man and mouse.
Mutations in the genes that encode filamin-1, Lis1 and doublecortin are responsible for X-linked lissencephaly in man, whereas mutations in the genes that encode Cdk5, its activator p35 and the reelin-signaling pathway disturb migration and architectonic development in mice. To understand the action of genes that control neuronal migration and the phenotype of corresponding defects, it might be as important to consider the positioning of the nucleus as it is to consider the guidance of the leading process.
Walsh
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Division of Neurogenetics, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Institutes of Medicine, Boston, Massachusetts 02115, USA. cwalsh@caregroup.harvard.edu
Goffinet
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D043203:1-Alkyl-2-acetylglycerophosphocholine Esterase; D000818:Animals; D001921:Brain / Q000002:abnormalities*; D000013:Congenital Abnormalities / Q000235:genetics*; D003285:Contractile Proteins / Q000235:genetics; D000089622:Doublecortin Domain Proteins; D000089662:Doublecortin Protein; D064448:Filamins; D006801:Humans; D051379:Mice; D008840:Microfilament Proteins / Q000235:genetics; D008869:Microtubule-Associated Proteins / Q000235:genetics; D009154:Mutation; D009474:Neurons / Q000502:physiology*; D009479:Neuropeptides / Q000235:genetics; D000091042:Reelin Protein; D014960:X Chromosome
D003285:Contractile Proteins; C000714847:Dcx protein, mouse; D000089622:Doublecortin Domain Proteins; D000089662:Doublecortin Protein; D064448:Filamins; D008840:Microfilament Proteins; D008869:Microtubule-Associated Proteins; D009479:Neuropeptides; D000091042:Reelin Protein; D043203:1-Alkyl-2-acetylglycerophosphocholine Esterase; C084102:PAFAH1B1 protein, human; C513190:Pafah1b1 protein, mouse; C000717127:RELN protein, human; C000717128:Reln protein, mouse