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

• Published in: Experimental Animals Vol. 56; no. 2; pp. 161 - 165
• Main Authors: GOHMA, Hiroshi, KURAMOTO, Takashi, MATALON, Reuben, SURENDRAN, Sankar, TYRING, Stephen, KITADA, Kazuhiro, SASA, Masashi, SERIKAWA, Tadao
• Format: Journal Article
• Language: English
• Published: Japan Japanese Association for Laboratory Animal Science 01-04-2007
• Subjects: Amidohydrolases - deficiency; Amidohydrolases - genetics; Animals; aspartoacylase; attractin; Electroencephalography; epilepsy; Epilepsy, Absence - genetics; Epilepsy, Absence - metabolism; Epilepsy, Absence - physiopathology; Female; Gene Silencing; Male; Membrane Proteins - deficiency; Membrane Proteins - genetics; Mice; Mice, Knockout; Mice, Mutant Strains; Seizures - genetics; Seizures - metabolism; Seizures - physiopathology
• ISSN: 1341-1357; 1881-7122
• DOI: 10.1538/expanim.56.161

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.