Absence-Like and Tonic Seizures in Aspartoacylase/Attractin Double-Mutant Mice

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  • GOHMA Hiroshi
    Institute of Laboratory Animals, Graduate School of Medicine, Kyoto University
  • KURAMOTO Takashi
    Institute of Laboratory Animals, Graduate School of Medicine, Kyoto University
  • MATALON Reuben
    Department of Pediatrics and Human Biological Chemistry and Genetics, Children's Hospital
  • SURENDRAN Sankar
    Department of Pediatrics and Human Biological Chemistry and Genetics, Children's Hospital
  • TYRING Stephen
    Department of Pediatrics and Human Biological Chemistry and Genetics, Children's Hospital
  • KITADA Kazuhiro
    Center for Advanced Science and Technology, Hokkaido University
  • SASA Masashi
    Nagisa Hospital
  • SERIKAWA Tadao
    Institute of Laboratory Animals, Graduate School of Medicine, Kyoto University

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The Spontaneously Epileptic Rat (SER), a double-mutant for tremor and zitter mutations, shows spontaneous occurrences of absence-like and tonic seizures. Several lines of evidence suggest that the combined effect of Aspa and Atrn mutations is the most likely cause of the epileptic phenotype of the SER. To address this issue, we produced a new double-mutant mouse line carrying both homozygous Aspa-knockout and Atrnmg-3J mutant alleles. The Aspa/Atrn double-mutant mice exhibited absence-like and tonic seizures that were characterized by the appearance of 5-7 Hz spike-wave-like complexes and low voltage fast waves on EEGs. These results demonstrate directly that the simultaneous loss of the Aspa and Atrn gene functions causes epileptic seizures in the mouse and suggest that both Aspa and Atrn deficiencies might be responsible for epileptic seizures in the SER.<br>

収録刊行物

  • Experimental Animals

    Experimental Animals 56 (2), 161-165, 2007

    公益社団法人 日本実験動物学会

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