{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T10:03:43Z","timestamp":1760954623291},"reference-count":33,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2003,10,21]],"date-time":"2003-10-21T00:00:00Z","timestamp":1066694400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2004,4,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In type I or classical lissencephaly, two genetic causes, namely the <jats:italic>LIS1<\/jats:italic> gene mapping at 17p13.3 and the <jats:italic>DCX<\/jats:italic> (doublecortin on X) gene mapping at Xq22.3 are involved. These are considered to act during corticogenesis on radial migratory pathways. The prevailing view is that heterozygous mutations in the <jats:italic>LIS1<\/jats:italic> gene and hemizygous mutations in the <jats:italic>DCX<\/jats:italic> gene produce similar histological pattern. The present detailed neuropathological study in two unrelated fetuses with respectively a mutation in the <jats:italic>LIS1<\/jats:italic> and the <jats:italic>DCX<\/jats:italic> genes do not confirm this view. In <jats:italic>LIS1<\/jats:italic> mutation, the cortical ribbon displays a characteristic inverted organization, also called \u201cfour layered cortex\u201d while in <jats:italic>DCX<\/jats:italic> mutation, the cortex displays a roughly ordered \u201csix layered\u201d lamination. Our hypothesis is that mutations of the <jats:italic>LIS1<\/jats:italic> and <jats:italic>DCX<\/jats:italic> genes, may not affect the same neuronal arrangement in the neocortex. Because the pathology of proven XLIS is rarely documented, further detailed neuropathological analysis in other cases identified through molecular study would be of a great help in the recognition of neuronal population involved in these migrational disorders and their underlying molecular mechanism. \u00a9 2003 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.a.20569","type":"journal-article","created":{"date-parts":[[2004,3,26]],"date-time":"2004-03-26T16:16:05Z","timestamp":1080317765000},"page":"123-128","source":"Crossref","is-referenced-by-count":28,"title":["Neocortical neuronal arrangement in LIS1 and DCX lissencephaly may be 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