| RRC ID |
33264
|
| 著者 |
Fumoto N, Mashimo T, Masui A, Ishida S, Mizuguchi Y, Minamimoto S, Ikeda A, Takahashi R, Serikawa T, Ohno Y.
|
| タイトル |
Evaluation of seizure foci and genes in the Lgi1(L385R/+) mutant rat.
|
| ジャーナル |
Neurosci Res
|
| Abstract |
Mutations in the leucine-rich, glioma inactivated 1 (LGI1) gene have been identified in patients with autosomal dominant lateral temporal lobe epilepsy (ADLTE). We previously reported that Lgi1 mutant rats, carrying a missense mutation (L385R) generated by gene-driven N-ethyl-N-nitrosourea (ENU) mutagenesis, showed generalized tonic-clonic seizures (GTCS) in response to acoustic stimuli. In the present study, we assessed clinically relevant features of Lgi1 heterozygous mutant rats (Lgi1(L385R/+)) as an animal model of ADLTE. First, to explore the focus of the audiogenic seizures, we performed electroencephalography (EEG) and brain Fos immunohistochemistry in Lgi1(L385R/+) and wild type rats. EEG showed unique seizure patterns (e.g., bilateral rhythmic spikes) in Lgi1(L385R/+) rats with GTCS. An elevated level of Fos expression indicated greater neural excitability to acoustic stimuli in Lgi1(L385R/+) rats, especially in the temporal lobe, thalamus and subthalamic nucleus. Finally, microarray analysis revealed a number of differentially expressed genes that may be involved in epilepsy. These results suggest that Lgi1(L385R/+) rats are useful as an animal model of human ADLTE.
|
| 巻・号 |
80
|
| ページ |
69-75
|
| 公開日 |
2014-3-1
|
| DOI |
10.1016/j.neures.2013.12.008
|
| PII |
S0168-0102(13)00290-3
|
| PMID |
24406746
|
| MeSH |
Acoustic Stimulation / adverse effects
Animals
Brain / metabolism
Disease Models, Animal
Electroencephalography
Epilepsy, Reflex / genetics*
Epilepsy, Reflex / pathology
Gene Expression Profiling
Gene Expression Regulation / genetics
Intracellular Signaling Peptides and Proteins
Mice, Transgenic
Mutation, Missense / genetics*
Oligonucleotide Array Sequence Analysis
Proteins / genetics*
Proto-Oncogene Proteins c-fos / metabolism
Rats
Rats, Inbred F344
|
| IF |
2.645
|
| 引用数 |
8
|
|
WOS 分野
|
NEUROSCIENCES
|
| リソース情報 |
| ラット |
F344-Lgi1m1Kyo (strainID=1036) |
