Neocortical neuronal arrangement in Miller Dieker syndrome.
Neocortical neuronal arrangement in Miller Dieker syndrome.
复制标题
米勒迪克综合征的新皮质神经元排列。
DOI：
10.1007/s00401-005-0010-3
复制
发表时间：
2006
影响因子：
12.7
通讯作者：
Walsh,ChristopherA
中科院分区：
文献类型：
--
作者：
Sheen,VolneyL;Ferland,RussellJ;Neal,Jason;Harney,Megan;Hill,RobertS;Banham,Alison;Brown,Phillip;Chenn,Anjen;Corbo,Joseph;Hecht,Jonathan;Folkerth,Rebecca;Walsh,ChristopherA
Miller Dieker syndrome (MDS, type I lissencephaly) is a neuronal migration disorder, which is caused by deletions along the short arm of chromosome 17 (17p13.3). Recent studies would suggest that the cortical lamination in MDS is inverted, based on morphological criteria. The present neuropathological study examines the cerebral cortex from a 33-week old fetus with MDS using both neuronal and laminar-specific markers. These expression studies demonstrate a relatively preserved cortex and cortical lamination, overlying a layer of immature neurons in MDS brain. The findings are consistent with both a migratory and proliferative defect, giving rise to lissencephaly. Moreover, characterization of such rare human malformations of cortical development by immunohistochemical techniques will provide a greater understanding of the underlying mechanisms.
