Journal
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
Volume 107, Issue 11, Pages 5076-5081Publisher
NATL ACAD SCIENCES
DOI: 10.1073/pnas.0908790107
Keywords
brain; lissencephaly; neuronal migration; nuclear envelope; nuclear lamina
Categories
Funding
- National Institutes of Health [AR050200, HL76839, HL86683, HL89781, GM66152]
- March of Dimes [6-FY2007-1012]
- Ellison Medical Foundation
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Nuclear lamins are components of the nuclear lamina, a structural scaffolding for the cell nucleus. Defects in lamins A and C cause an array of human diseases, including muscular dystrophy, lipodystrophy, and progeria, but no diseases have been linked to the loss of lamins B1 or B2. To explore the functional relevance of lamin B2, we generated lamin B2-deficient mice and found that they have severe brain abnormalities resembling lissencephaly, with abnormal layering of neurons in the cerebral cortex and cerebellum. This neuronal layering abnormality is due to defective neuronal migration, a process that is dependent on the organized movement of the nucleus within the cell. These studies establish an essential function for lamin B2 in neuronal migration and brain development.
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