Abnormal development of the cerebral cortex and cerebellum in the setting of lamin B2 deficiency.
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.
Proceedings of the National Academy of Sciences of the United States of America
March 16, 2010
Agency: NIAMS NIH HHS, Id: AR050200
Agency: NIGMS NIH HHS, Id: GM66152
Agency: NHLBI NIH HHS, Id: HL76839
Agency: NHLBI NIH HHS, Id: HL86683
Agency: NHLBI NIH HHS, Id: HL89781
Agency: NIA NIH HHS, Id: R01 AG035626
Agency: NIA NIH HHS, Id: R01 AG035626-07
Lamin Type B
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