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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.

Pubmed ID: 20145110

Authors

  • Coffinier C
  • Chang SY
  • Nobumori C
  • Tu Y
  • Farber EA
  • Toth JI
  • Fong LG
  • Young SG

Journal

Proceedings of the National Academy of Sciences of the United States of America

Publication Data

March 16, 2010

Associated Grants

  • 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

Mesh Terms

  • Animals
  • Cell Movement
  • Cerebellum
  • Cerebral Cortex
  • Gene Silencing
  • Lamin Type B
  • Mice
  • Neurons