Journal
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY
Volume 21, Issue 8, Pages 823-830Publisher
ACADEMIC PRESS LTD- ELSEVIER SCIENCE LTD
DOI: 10.1016/j.semcdb.2010.07.008
Keywords
Neurogenetic disease; Neuronal migration; Neurogenesis; Lissencephaly; Therapy
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Funding
- NICHD NIH HHS [R01 HD047380] Funding Source: Medline
- NINDS NIH HHS [R01 NS041030] Funding Source: Medline
- EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH &HUMAN DEVELOPMENT [R01HD047380] Funding Source: NIH RePORTER
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS041030] Funding Source: NIH RePORTER
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Lissencephaly is a severe human neuronal migration defect characterized by a smooth cerebral surface, mental retardation and seizures. The two most common genes mutated in patients with lissencephaly are LIS1 and DCX. LIS1 was the first gene cloned that was important for neuronal migration in any organism, and heterozygous mutations or deletions of LIS1 are found in the majority of patients with lissencephaly, while DCX mutations were found in males with X-linked lissencephaly. In this review, we will discuss how an understanding of the molecular and cellular pathways disrupted in model organisms with Lis1 and Dcx mutations or knock-down not only provide insights into the normal processes of neuronal migration, including neurogenesis, but they also may lead to potential novel therapeutic strategies for these severe cortical malformations. (C) 2010 Elsevier Ltd. All rights reserved.
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