Impaired proliferation and migration in human Miller-Dieker neural precursors.

Annals of Neurology
Volney L SheenChristopher A Walsh

Abstract

Miller-Dieker syndrome (MDS) is a malformation of cortical development that results in lissencephaly (meaning smooth brain). This disorder is caused by heterozygous deletions on chromosome 17p13.3, including the lissencephaly 1 (LIS1) gene. Various mouse models have been used as an experimental paradigm in understanding human lissencephaly, but clear limitations exist in these studies, particularly because mice are naturally lissencephalic. Thus, the objective of this article was to establish human neural precursor cell lines from postmortem MDS tissue and to characterize the pathological cellular processes that contribute to the human lissencephalic phenotype. Human neural precursors were isolated and expanded from the frontal cortices of a 33-week postmortem fetus with MDS and an age-matched control subject. Relative rates of proliferation and cell death were assessed in vitro, whereas the migration of precursors was examined after transplantation in vivo. Precursors showed haploinsufficiency of the LIS1 gene and a reduction in LIS1 protein. Precursors could also differentiate into both neurons and glia. MDS precursors demonstrated impairments in neuronal migration, diminished rates of cell proliferation, and increased cell d...Continue Reading

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Feb 4, 2006·Acta Neuropathologica·Volney L SheenChristopher A Walsh

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Citations

Nov 4, 2010·Acta Neuropathologica·Gaëlle FriocourtAnnie Laquerrière
Jun 24, 2009·The Cerebellum·Donald Bryant CameronHitoshi Komuro
Oct 9, 2007·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Jaime Imitola
May 13, 2008·Human Molecular Genetics·Ashley S PawliszYuanyi Feng
Nov 14, 2006·Current Opinion in Pediatrics·Gewei Lian, Volney Sheen
May 13, 2008·The Neurologist·Trudy PangVolney Sheen
Jun 1, 2012·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Gewei LianVolney L Sheen
Jun 10, 2011·Journal of Visualized Experiments : JoVE·Jie LuVolney L Sheen
Sep 26, 2009·Seminars in Pediatric Neurology·Ganeshwaran H Mochida
Jan 19, 2007·Journal of Anatomy·Jason NealVolney L Sheen
Sep 14, 2007·Clinical Genetics·Elisabetta LenziniVanna Pecile
Apr 4, 2008·Developmental Medicine and Child Neurology·Salvatore GrossoPaolo Balestri
Jun 15, 2007·American Journal of Human Genetics·Mark O'DriscollPenny A Jeggo
Nov 22, 2019·Frontiers in Cellular Neuroscience·Maria Eleni KastritiDomna Karagogeos
Jul 12, 2019·Frontiers in Neuroscience·Evelyn ChukwurahSofia B Lizarraga

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