Astrocyte-specific TSC1 conditional knockout mice exhibit abnormal neuronal organization and seizures

Ann Neurol. 2002 Sep;52(3):285-96. doi: 10.1002/ana.10283.

Abstract

Persons affected with tuberous sclerosis complex (TSC) develop a wide range of neurological abnormalities including aberrant neuronal migration and seizures. In an effort to model TSC-associated central nervous system abnormalities in mice, we generated two independent lines of astrocyte-specific Tsc1 conditional knockout mice by using the Cre-LoxP system. Astrocyte-specific Tsc1-null mice exhibit electroencephalographically proven seizures after the first month of age and begin to die at 3 to 4 months. Tsc1-null mice show significant increases in astrocyte numbers throughout the brain by 3 weeks of age and abnormal neuronal organization in the hippocampus between 3 and 5 weeks. Moreover, cultured Tsc1-null astrocytes behave similar to wild-type astrocytes during log phase growth but demonstrate increased saturation density associated with reduced p27(Kip1) expression. Collectively, our results demonstrate that astrocyte-specific disruption of Tsc1 in mice provides a context-dependent growth advantage for astrocytes that results in abnormalities in neuronal organization and epilepsy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Astrocytes / chemistry
  • Astrocytes / pathology*
  • Cell Cycle Proteins / genetics
  • Cell Division
  • Cells, Cultured
  • Cyclin-Dependent Kinase Inhibitor p27
  • Electroencephalography
  • Epilepsy / genetics
  • Epilepsy / pathology*
  • Gliosis / pathology
  • Hippocampus / abnormalities*
  • Hippocampus / pathology
  • In Vitro Techniques
  • Mice
  • Mice, Knockout
  • Neurons / pathology*
  • Proteins / analysis
  • Proteins / genetics*
  • Tuberous Sclerosis Complex 1 Protein
  • Tumor Suppressor Proteins / genetics

Substances

  • Cdkn1b protein, mouse
  • Cell Cycle Proteins
  • Proteins
  • Tsc1 protein, mouse
  • Tuberous Sclerosis Complex 1 Protein
  • Tumor Suppressor Proteins
  • Cyclin-Dependent Kinase Inhibitor p27