Strains

Japanese

NBRP Rat No: 0455

Strain Name: F344-Scn1a^m1Kyo

Commmon Name: F344/NSlc-Scn1aKyo811

Principal Investigator

Tadao Serikawa

Organization

Graduate School of Medicine, Kyoto University Institute of Laboratory Animals

Address

Yoshidakonoe-cho, Sakyo-ku

606-8501 Kyoto

JAPAN

Telephone

075-753-4360

Fax: 075-753-4409

serikawa@anim.med.kyoto-u.ac.jp

Inbred Generations

N11F5 (April 2012)

Coat Color

Deposition Status

albino (c)

Embryo Sperm Live Animals

Usage Restrictions

The recipient of BIOLOGICAL RESOURCE shall obtain a prior written consent on use of it from the DEPOSITOR.
For a commercial use of this resource, a new contract must be concluded between the depositor and the recipient.

Genetic Status

Inbred

Segregating

Congenic

Consomic

Recombinant

Coisogenic

Spont. Mutant

Transgene

Ind. Mutant

Others

Comercial Availability

Research Category

Diabetes Obesity

Neurobiology

Ophthalmology

Dentistry

Cardio- Hypertension

Oncology

Metabolism

Otorhinology

Immunology

Infectious Disease

Osteology

Internal Medicine

Dermatology

Reproduction

Development

Behavior

Hematology

Urology

Pharmacology

Others

Control Strains

Reporter gene Strains

Gene

Scn1a: sodium channel, voltage-gated, type I, alpha

Origin

This strain was established by ENU mutagenesis (gene-driven). This strain possesses a point mutation in the Scn1a gene (N1417H) (Mashimo, 2010). Hyperthermia-induced seizure-susceptible (Hiss) rat. (May 26, 2010)

Strain Characteristics

A point mutation in Scn1a gene.
This strain possesses a missense mutation (N1417H) in the third pore region of the sodium channel, Scn1a. Despite their normal appearance under ordinary circumstances, Scn1a mutant rats exhibited remarkably high susceptibility to hyperthermia-induced seizures. Scn1a is a causative gene for human GEFS+. Immunohistochemical analysis revealed that hyperthermic seizures induced a widespread elevation of Fos-immunoreactivity in the cerebral cortices and limbic regions (Ohno, 2010). This rat showed a significantly lower threshold in epileptiform discharges in response to local stimulation of the hippocampus. Diazepam and sodium valproate are effective to hyperthermic seizure of this rat (Ohno, 2010). (Oct 11, 2010)

Breeding Conditions

Good breeding performance.

Genotyping

<a href="http://www.anim.med.kyoto-u.ac.jp/nbr/documents/PCR_Gene/Scn1a_m1kyo_en.pdf">Genotyping protocol for　Scn1am1Kyo</a>

References

Serikawa T, Mashimo T, Kuramoto T, Voigt B, Ohno Y, Sasa M.
Advances on Genetic Rat Models of Epilepsy.
Exp Anim. 2015 Feb 5;64(1):1-7.

Ohmori I, Kawakami N, Liu S, Wang H, Miyazaki I, Asanuma M, Michiue H, Matsui H, Mashimo T, Ouchida M.
Methylphenidate improves learning impairments and hyperthermia-induced seizures caused by an Scn1a mutation.
Epilepsia. 2014 Oct;55(10):1558-67.

Ohmori I, Hayashi K, Wang H, Ouchida M, Fujita N, Inoue T, Michiue H, Nishiki T, Matsui H.
Inhalation of 10% carbon dioxide rapidly terminates Scn1a mutation-related hyperthermia-induced seizures.
Epilepsy Res. 2013 Jul;105(1-2):220-4.

Ohno Y, Ishihara S, Mashimo T, Sofue N, Shimizu S, Imaoku T, Tsurumi T, Sasa M, Serikawa T.
Scn1a missense mutation causes limbic hyperexcitability and vulnerability to experimental febrile seizures.
Neurobiol Dis. 41(2):261-269, 2011.

Hayashi K, Ueshima S, Ouchida M, Mashimo T, Nishiki T, Sendo T, Serikawa T, Matsui H, Ohmori I.
Therapy for hyperthermia-induced seizures in Scn1a mutant rats.
Epilepsia 52(5):1010-1017, 2011

Ohno Y, Sofue N, Ishihara S, Mashimo T, Sasa M, Serikawa T.
Scn1a missense mutation impairs GABA(A) receptor-mediated synaptic transmission in the rat hippocampus.
Biochem Biophys Res Commun. 2010 Sep 10;400(1):117-22.

Mashimo T, Ohmori I, Ouchida M, Ohno Y, Tsurumi T, Miki T, Wakamori M, Ishihara S, Yoshida T, Takizawa A, Kato M, Hirabayashi M, Sasa M, Mori Y, Serikawa T.
A missense mutation of the gene encoding voltage-dependent sodium channel (Nav1.1) confers susceptibility to febrile seizures in rats.
J Neurosci. 2010 Apr 21;30(16):5744-53.