Abstract：
:This study aimed to examine the effects of Cacna1a mutation on the phenotype of Scn1a-associated epilepsy in rats. We used rats with an N1417H missense mutation in the Scn1a gene and others with an M251K mutation in the Cacna1a gene. Scn1a/Cacna1a double mutant rats were generated by mating both Scn1a and Cacna1a mutants. We investigated general health and the epileptic phenotype in all these genotypes. The onset threshold of hyperthermia-induced seizures was examined at 5 weeks and spontaneous seizures were monitored using video-EEG recordings from 6 to 12 weeks of age. Scn1a/Cacna1a double mutants showed significantly reduced threshold for hyperthermia-sensitive seizures onset compared with the Scn1a mutants and had absence seizures having 6-7 c/s spike-wave bursts with changes in the spike-wave pattern, whereas Cacna1a mutants had regular 6-7 c/s spike-wave bursts. In Scn1a/Cacna1a double mutants, 6-7 c/s spike-wave bursts were accompanied with eyelid myoclonia and continuously shifting generalized clonic seizures, which were not observed in either Scn1a or Cacna1a mutants. Although a curvature of the spine was observed in rats of all these genotypes, the degree of curvature was more pronounced in Scn1a/Cacna1a double mutants, followed by Cacna1a and Scn1a mutants. Our results indicate that Cacna1a and Scn1a mutations mutually alter their original phenotypes in rats. The phenotype of absence seizures with eyelid myoclonia, generalized clonic seizures, and of spine curvature in the Scn1a/Cacna1a double mutants were similar to that observed in patients with Dravet syndrome.
journal_name
Neurochem Intjournal_title
Neurochemistry internationalauthors
Ohmori I,Kobayashi K,Ouchida Mdoi
10.1016/j.neuint.2020.104859subject
Has Abstractpub_date
2020-12-01 00:00:00pages
104859eissn
0197-0186issn
1872-9754pii
S0197-0186(20)30250-3journal_volume
141pub_type
杂志文章abstract：:The present study was carried out to clarify whether the imidazolines clonidine, moxonidine and cirazoline as well as the guanidine aganodine inhibit noradrenaline release in the rat and rabbit brain via imidazoline receptors, alpha 2-adrenoceptors and/or histamine H3 receptors. Slices or synaptosomes from the rat or ...
journal_title：Neurochemistry international
pub_type： 杂志文章
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abstract：:The distribution of substance P in the colon of piebald lethal (s(1)/s(1)) mice was studied by radioimmunoassay and immunohistochemistry. These animals inherit as a Mendelian recessive trait an aganglionic distal colon. In the region proximal to the aganglionic segment, there is an extensive transitional or hypogangli...
journal_title：Neurochemistry international
pub_type： 杂志文章
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journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(89)90164-2
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journal_title：Neurochemistry international
pub_type： 杂志文章,评审
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journal_title：Neurochemistry international
pub_type： 杂志文章,评审
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journal_title：Neurochemistry international
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journal_title：Neurochemistry international
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journal_title：Neurochemistry international
pub_type： 杂志文章,评审
doi：10.1016/j.neuint.2014.06.002
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journal_title：Neurochemistry international
pub_type： 杂志文章
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journal_title：Neurochemistry international
pub_type： 杂志文章,评审
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journal_title：Neurochemistry international
pub_type： 杂志文章
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journal_title：Neurochemistry international
pub_type： 杂志文章
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更新日期：1992-01-01 00:00:00
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journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2008.12.003
更新日期：2009-03-01 00:00:00
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journal_title：Neurochemistry international
pub_type： 杂志文章,评审
doi：10.1016/j.neuint.2018.10.013
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journal_title：Neurochemistry international
pub_type： 杂志文章,评审
doi：10.1016/j.neuint.2006.03.018
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journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(80)90075-3
更新日期：1980-01-01 00:00:00
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journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(84)90072-x
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journal_title：Neurochemistry international
pub_type： 杂志文章
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journal_title：Neurochemistry international
pub_type： 杂志文章
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journal_title：Neurochemistry international
pub_type： 杂志文章
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journal_title：Neurochemistry international
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journal_title：Neurochemistry international
pub_type： 杂志文章
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journal_title：Neurochemistry international
pub_type： 杂志文章
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journal_title：Neurochemistry international
pub_type： 杂志文章
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更新日期：1997-04-01 00:00:00
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journal_title：Neurochemistry international
pub_type： 杂志文章,评审
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journal_title：Neurochemistry international
pub_type： 杂志文章
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journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2008.12.013
更新日期：2009-05-01 00:00:00
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journal_title：Neurochemistry international
pub_type： 杂志文章,评审
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abstract：:Amyloid β (Aβ) oligomers are presumed to be one of the causes of Alzheimer's disease (AD). Previously, we identified the E693Δ mutation in amyloid precursor protein (APP) in patients with AD who displayed almost no signals of amyloid plaques in amyloid imaging. We generated APP-transgenic mice expressing the E693Δ mut...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2012.05.018
更新日期：2012-08-01 00:00:00
abstract：:gamma-Aminobutyric acid (GABA) levels and the activity of glutamate decarboxylase were measured in homogenates of rat brain cortical tissue, at different times after chronic intracortical infusion of GABA in vivo during 2, 6 or 24 h. Cortical electrical activity was also recorded. As previously described, about 1 h af...
journal_title：Neurochemistry international
pub_type： 杂志文章
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