The proposed program project (PP) will examine the basis of neuronal migration in the forebrain. The focal point of the PP will be the study of the molecular basis of classical lissencephaly and subcortical band heterotopia, which are now recognized to constitute a spectrum of brain malformations whose fundamental morphologic phenotype reflects disordered migration of cortical neurons. Recent data, derived from the laboratories of the PP investigators, have defined two specific molecular defects as the basis for these distinct malformations (LIS1 on chromosome 17p13.3 and XLIS/DBCN on Xq22.3-q23). The clinical consequences can be very substantial -- intractable epilepsy and severe mental retardation. The proposed PP, which emanates from the close collaborative efforts of the PP investigators over the past 10-15 years, consists of four individual projects and three Cores. Projects 1 and 2 deal mainly with LIS1 and Projects 3 and 4 mainly with XLIS/DBCN. Cores A and B are developed to provide the clinical and diagnostic services for Projects 1,3, and 4. Core C will provide transgenic animals for Projects 2, 3, and 4.

Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Program Projects (P01)
Project #
3P01NS039404-04S1
Application #
6662964
Study Section
Special Emphasis Panel (ZNS1 (01))
Program Officer
Finkelstein, Robert
Project Start
1999-04-05
Project End
2004-03-31
Budget Start
2002-04-01
Budget End
2003-03-31
Support Year
4
Fiscal Year
2002
Total Cost
$10,000
Indirect Cost
Name
University of Chicago
Department
Genetics
Type
Schools of Medicine
DUNS #
225410919
City
Chicago
State
IL
Country
United States
Zip Code
60637
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