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Development of preventative measures against epilepsy using novel model animals (kick-in)

Research Project

Project/Area Number 23659529
Research Category

Grant-in-Aid for Challenging Exploratory Research

Allocation TypeMulti-year Fund
Research Field Pediatrics
Research InstitutionFukuoka University

Principal Investigator

HIROSE Shinichi  福岡大学, 医学部, 教授 (60248515)

Co-Investigator(Renkei-kenkyūsha) TAKANO Yukio  福岡大学, 薬学部, 教授 (50113246)
SAITO Ryo  福岡大学, 薬学部, 講師 (80122696)
KATSURABAYASHI Shutaro  福岡大学, 薬学部, 助教 (50435145)
Project Period (FY) 2011 – 2012
Project Status Completed (Fiscal Year 2012)
Budget Amount *help
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2012: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2011: ¥2,860,000 (Direct Cost: ¥2,200,000、Indirect Cost: ¥660,000)
Keywordsモデル動物 / フロセミド / てんかん
Research Abstract

Dravet syndrome results from various mutations of SCN1A, the gene encoding a1 subunit of Nav1.1 channel. Microdeletion of chromosome including SCN1A leads the most severe phenotype of Dravet syndrome. We have successfully genetically engineered mice bearing a heterozygous microdeletion of Scn1a, the mouse homologue of human SCN1A. The mice exhibited severe convulsions and epileptic discharges on Electro corticogram during 4 to 8 weeks of age. We are now examining effects of NKCC1 inhibitors on the development of the phenotypes whether the inhibitors can exert a prophylactic effect. Also, we are examining the neuro-electrophysiological characteristics of the mice using the slice patch clump method on brain. Primary culture of neurons of the mice are also has been implemented.(金額単位:円)直接経費 間接経費 合 計

Report

(3 results)
  • 2012 Annual Research Report   Final Research Report ( PDF )
  • 2011 Research-status Report
  • Research Products

    (43 results)

All 2013 2012 2011 2010 2009 Other

All Journal Article (19 results) (of which Peer Reviewed: 19 results) Presentation (21 results) (of which Invited: 2 results) Book (2 results) Remarks (1 results)

  • [Journal Article] Properties of a novel GABAA receptor gamma2 subunit mutation associated with seizures.2013

    • Author(s)
      Migita K, Yamada J, Nikaido Y, Shi X, Kaneko S, Hirose S, Ueno S.
    • Journal Title

      J Pharmacol Sci.

      Volume: 121(1) Pages: 84-7

    • Related Report
      2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Identification of ATP1A3 mutations by exome sequencing as the cause of alternating hemiplegia of childhood in Japanese patients.2013

    • Author(s)
      Ishii A, Saito Y, Mitsui J, Ishiura H, Yoshimura J, Arai H, Yamashita S, Kimura S, Oguni H, Morishita S, Tsuji S, Sasaki M, Hirose S.
    • Journal Title

      PLoS One

      Volume: 8 Issue: 2 Pages: e56120-e56120

    • DOI

      10.1371/journal.pone.0056120

    • Related Report
      2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] A Novel Mutation of Human Liver Alanine:Glyoxylate Aminotransferase Causes Primary Hyperoxaluria Type 1: Immunohistochemical Quantification and Subcellular Distribution2012

    • Author(s)
      Kawai C, Minatogawa Y, Akiyoshi H, Hirose S, Suehiro T, Tone S
    • Journal Title

      ACTA HISTOCHEMICA ET CYTOCHEMICA

      Volume: 45 Issue: 2 Pages: 121-129

    • DOI

      10.1267/ahc.11042

    • NAID

      130001854237

    • ISSN
      0044-5991, 1347-5800
    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] On the likelihood of SCN1A microdeletions or duplications in Dravet syndrome withmissense mutation.2012

    • Author(s)
      Shi X, Wang J, Kurahashi H, Ishii A, Higurashi N, Kaneko S, Hirose S.
    • Journal Title

      Brain Dev

      Volume: 34(8) Pages: 617-9

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Mutations of the SCN1A gene in acute encephalopathy2012

    • Author(s)
      Saitoh M, Shinohara M, Hoshino H,Kubota M, Amemiya K, Takanashi JL, Hwang SK,Hirose S, Mizuguchi M
    • Journal Title

      Epilepsia

      Volume: 53 Issue: 3 Pages: 558

    • DOI

      10.1111/j.1528-1167.2011.03402.x

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Mutations in PRRT2 responsible for paroxysmal kinesigenic dyskinesias also cause benign familialinfantile convulsions.2012

    • Author(s)
      Ono S, Yoshiura K , KinoshitaA, Kikuchi T, Nakane Y, Kato N,Sadamatsu M , Konishi T , Nagamitsu S , Matsuura M, Yasuda A, KomineM, Kanai K , Inoue T , Osamura T , Saito K , Hirose S, Koide H, Tomita H , Ozawa H , Niikawa N and Kurotaki N.
    • Journal Title

      J Hum Genet

      Volume: 57 Issue: 5 Pages: 1-4

    • DOI

      10.1038/jhg.2012.23

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] PCDH19 Mutation in Japanese Females with Epilepsy2012

    • Author(s)
      Higurashi N, Shi X, Yasumoto S, Oguni K, Itomi K, Miyamoto A, Shirishi H, Kato T, Makita Y, Hirose S.
    • Journal Title

      Epilepsy Res

      Volume: 99 Issue: 1-2 Pages: 28-37

    • DOI

      10.1016/j.eplepsyres.2011.10.014

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Prevalence of SCN1A mutations in children with suspected Dravet syndrome and intractable childhood epilepsy.2012

    • Author(s)
      Wang JW, Shi XY, Kurahashi H, Hwang SK, Ishii A, Higurashi N, Kaneko S, Hirose S,Epilepsy Genetic Study Group J.
    • Journal Title

      Epilepsy Res.

      Volume: 102(3) Issue: 3 Pages: 195-200

    • DOI

      10.1016/j.eplepsyres.2012.06.006

    • Related Report
      2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Clinical spectrum of SCN2A mutations.2012

    • Author(s)
      Shi X, Yasumoto S, Kurahashi H, Nakagawa E, Fukasawa T, Uchiya S, Hirose S.
    • Journal Title

      Brain Dev.

      Volume: 34(7) Pages: 541-5

    • Related Report
      2012 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Clinical spectrum of SCN2A mutations2011

    • Author(s)
      Shi X, Yasumoto S, Kurahashi K,Nakagawa E, Fukasawa T, Uchiya S, Hirose S
    • Journal Title

      Brain Dev

      Volume: 34(7) Issue: 7 Pages: 541-5

    • DOI

      10.1016/j.braindev.2011.09.016

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] A boy with a severe phenotype of succinic semialdehyde dehydrogenase deficiency2011

    • Author(s)
      Yamakawa Y, Nakazawa T, Ishida A, Saito N, Komatsu M, Matsubara T, Obinata K, Hirose S, Okumura A, Shimizu T.
    • Journal Title

      Brain Dev

      Volume: 34(2) Issue: 2 Pages: 107-12

    • DOI

      10.1016/j.braindev.2011.05.003

    • NAID

      10031049740

    • Related Report
      2012 Annual Research Report 2012 Final Research Report 2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Early-onset absence epilepsy at eight months of age.2011

    • Author(s)
      Kobayashi Y, Akasaka N, Ohashi T,Saitoh S, Tomonoh Y, Hirose S, Tohyama J.
    • Journal Title

      Epileptic Disord

      Volume: 13(4) Pages: 417-21

    • Related Report
      2012 Final Research Report 2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Autosomal dominant nocturnal frontal lobe epilepsy: a genotypic comparative study of Japanese and Korean families carrying the CHRNA4 Ser284Leu mutation.2011

    • Author(s)
      Hwang SK, Makita Y, Kurahashi H, Cho YW, Hirose S
    • Journal Title

      J Hum Genet

      Volume: 56(8) Issue: s2 Pages: 609-12

    • DOI

      10.1111/j.1528-1167.2011.03002.x

    • NAID

      10030660618

    • Related Report
      2012 Final Research Report 2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Retrospective multiinstitutional study of the prevalence of early death in Dravet syndrome2011

    • Author(s)
      Sakauchi M,Oguni H, Kato I, Osawa M, Hirose S, Kaneko S, Takahashi Y, Takayama R, Fujiwara T
    • Journal Title

      Epilepsia

      Volume: 52(6) Issue: 6 Pages: 1144-9

    • DOI

      10.1111/j.1528-1167.2011.03053.x

    • Related Report
      2012 Final Research Report 2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Diagnosing nocturnal frontal lobe epilepsy: A case study of two children2011

    • Author(s)
      Tomonoh Y, Yasumoto S, Ihara Y, Fujita T, Nakamura N, Ninomiya S, Kodama R, Ideguchi H, Inoue T, Mitsudome A, Hirose S
    • Journal Title

      Seizure

      Volume: 20(7) Issue: 7 Pages: 583-5

    • DOI

      10.1016/j.seizure.2011.03.004

    • Related Report
      2012 Final Research Report 2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] The Developmental Changes of Na(v)1.1 and Na(v)1.2 Expression in the Human Hippocampus and Temporal Lobe.2011

    • Author(s)
      Wang W, Takashima S, Segawa Y, Itoh M, Shi X, Hwang SK, Nabeshima K, Takeshita M, Hirose S.
    • Journal Title

      Brain Res

      Volume: 1389(5) Pages: 61-70

    • DOI

      10.1016/j.brainres.2011.02.083

    • Related Report
      2012 Final Research Report 2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Positive association between benign familial infantile convulsions and LGI42010

    • Author(s)
      Ishii A, Zhang B, Kaneko S, Hirose S
    • Journal Title

      Brain Dev

      Volume: 32 Issue: 7 Pages: 538-43

    • DOI

      10.1016/j.braindev.2009.09.006

    • NAID

      10027491923

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Genetic testing in the epilepsies2010

    • Author(s)
      Ottman R, Hirose S
    • Journal Title

      Report of the ILAE Genetics Commission Epilepsia

      Volume: 51(4) Issue: 4 Pages: 655-670

    • DOI

      10.1111/j.1528-1167.2009.02429.x

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Journal Article] Lack of potassium current in W309R mutant KCNQ3 channel causing benign familial neonatalconvulsions (BFNC).2009

    • Author(s)
      Sugiura Y, Nakatsu F, Hiroyasu K, Ishii A, Hirose S, Okada M, Jibiki I, Ohno H, Kaneko S, Ugawa Y.
    • Journal Title

      Epilepsy Res

      Volume: 84(1) Issue: 1 Pages: 82-5

    • DOI

      10.1016/j.eplepsyres.2008.12.003

    • Related Report
      2012 Final Research Report
    • Peer Reviewed
  • [Presentation] Genetic analysis of Alternating Childhood2012

    • Author(s)
      Ishii A,saito Y,Sasaki M,Hirose S.
    • Organizer
      10th European Congress of Epileptology
    • Place of Presentation
      London,UK
    • Year and Date
      2012-09-30
    • Related Report
      2012 Final Research Report
  • [Presentation] Genetic analysis of PRRT2 for benign infantile epilepsy, infantile convulsions with choleoathetosis syndrom,and benign convulsions with mild gastroenteritis2012

    • Author(s)
      Ishii A,Yasumoto S,Ihara Y, Inoue T,Fujita T,Nakamura N,Ohfu M,Lee WT,Kaneko S, Hirose S.
    • Organizer
      2012Korea Epilepsy congress
    • Place of Presentation
      Incheon,Korea
    • Year and Date
      2012-06-08
    • Related Report
      2012 Final Research Report
  • [Presentation] Identification of ATP1A3 mutations by exome sequencing as the cause of AHC in japanese patients2012

    • Author(s)
      Ishii A,Hirose S.
    • Organizer
      symposium ATP1A3 in disease from gene mutations to new treatments
    • Place of Presentation
      Brussels,Belglum-
    • Related Report
      2012 Final Research Report
  • [Presentation] Genetics of benign neonatal seizures2012

    • Author(s)
      Hirose S.
    • Organizer
      12th international child Neurology Congress of Child Nuerology
    • Place of Presentation
      Brisbane,Australia
    • Related Report
      2012 Final Research Report
  • [Presentation] Neutral Hyperexcitability and the Effects of KCNQ Channel Openers on Pentylenetetrazole Induced Seizures on Mice in BFNS2012

    • Author(s)
      Tomonoh Y,Saito R,Araki K,Deshimaru M,Takano Y,Hirose S.
    • Organizer
      12th international child Neurology Congress of Child Nuerology
    • Place of Presentation
      Brisbane,Australia
    • Related Report
      2012 Final Research Report
  • [Presentation] Genetics of benign neonatal seizures2012

    • Author(s)
      Hirose S.
    • Organizer
      12th international Child Neurology Congress/ 11th Asian and Oceanian congress of Child Neurology
    • Place of Presentation
      Brisbane, Australia
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] Neural Hyperexcitability and the Effects of KCNQChannel Openers on Pentylenetetrazole Induced Seizures on Model Mice in BFNS2012

    • Author(s)
      Tomonoh Y, Saito R, Araki K, Deshimaru M,Takano Y,Hirose s.
    • Organizer
      12th International Child Neurology Congress/11th Asian and Oceanian Congress of Child Neurology
    • Place of Presentation
      Brisbane, Australia
    • Related Report
      2012 Annual Research Report
  • [Presentation] Identification of ATP1A3 mutations by exome sequencing as the cause of AHC in japanese patients2012

    • Author(s)
      Ishii A, Hirose S.
    • Organizer
      Symposium ATP1A3 in disease from gene mutations to new treatments
    • Place of Presentation
      Brussels, Belglum
    • Related Report
      2012 Annual Research Report
    • Invited
  • [Presentation] Autosomal Dominant nocturnal frontal Lobe Epilepsy:A Genotypic Comparative Study of Japanese and Korean Families Carrying the CHRNA4Ser284LeuMutation2011

    • Author(s)
      Su-Kyeong Hwang,Makita Y,Kurahashi H, Yong-Won Cho, Hirose S.
    • Organizer
      KES 韓国てんかん学会
    • Place of Presentation
      韓国
    • Year and Date
      2011-06-24
    • Related Report
      2012 Final Research Report
  • [Presentation] Guideline for genetic diagnosis for epilepsy2011

    • Author(s)
      Hirose s.
    • Organizer
      KES 韓国てんかん学会
    • Place of Presentation
      韓国
    • Year and Date
      2011-06-24
    • Related Report
      2012 Final Research Report
  • [Presentation] Update for genetics of epilepsy Basic Science Session2011

    • Author(s)
      Hirose S.
    • Organizer
      KES韓国てんかん学会
    • Place of Presentation
      韓国
    • Year and Date
      2011-06-24
    • Related Report
      2012 Final Research Report
  • [Presentation] The development of the DNA chip which aimed at the clinical application in epilepsy2011

    • Author(s)
      Sugawara T,Yoshida S, Wda K, Hirose S, Iwasa H,Kaneko S.
    • Organizer
      29th International Epilepsy Congress.
    • Place of Presentation
      Rome
    • Related Report
      2012 Final Research Report
  • [Presentation] Steroid pulse therapy as an effective treatment for refractory epilepsy in children with glutamate receptor (GLUR) antibodies2011

    • Author(s)
      Yasumoto S, Ihara Y, Fujita T, Tomonoh Y, Ninomiya S, Nakamura N, Ideguchi H, Inoue T, Takahashi Y, Hirose S.
    • Organizer
      29thInternational Epilepsy Congress
    • Place of Presentation
      Rome
    • Related Report
      2012 Final Research Report
  • [Presentation] Aoutosomal Dominant Nocturnal Frontal Lobe Epilepsy: A Genotypic Comparative Study of Japanese and Korean Families Carrying the CHRNA4 Ser284Leu Mutation2011

    • Author(s)
      Su-Kyeong Hwang Makita Y., Kurahashi H., Yong-Won Cho, Hirose S
    • Organizer
      KES 韓国てんかん学会
    • Place of Presentation
      韓国
    • Related Report
      2011 Research-status Report
  • [Presentation] The development of the DNA chip which aimed at the clinical application in epilepsy.2011

    • Author(s)
      Sugawara T., Yoshida S., Wada K., Hirose S., Iwasa H., Kaneko S.
    • Organizer
      29th International Epilepsy Congress
    • Place of Presentation
      Roma
    • Related Report
      2011 Research-status Report
  • [Presentation] Steroid pulse therapy as an effective treatment for refractory epilepsy in children with glutamate receptor (GluR) antibodies.2011

    • Author(s)
      Yasumoto S., Ihara Y., Fujita T.,Tomonoh Y., Ninomiya S., Nakamura N., Ideguchi H.,Inoue T.,Takahashi Y.,Hirose S.
    • Organizer
      29th International Epilepsy Congress
    • Place of Presentation
      Roma
    • Related Report
      2011 Research-status Report
  • [Presentation] Guideline for genetic diagnosis for epilepsy2011

    • Author(s)
      Hirose S.
    • Organizer
      KES 韓国てんかん学会(招待講演)
    • Place of Presentation
      韓国
    • Related Report
      2011 Research-status Report
  • [Presentation] Update for genetics of epilepsy plenary session2011

    • Author(s)
      Hirose S.
    • Organizer
      KES 韓国てんかん学会(招待講演)
    • Place of Presentation
      韓国
    • Related Report
      2011 Research-status Report
  • [Presentation] "Genetically engineered animal models for epilepsy " Basic Science Session2011

    • Author(s)
      Hirose S.
    • Organizer
      KES 韓国てんかん学会(招待講演)
    • Place of Presentation
      韓国
    • Related Report
      2011 Research-status Report
  • [Presentation] Genetic analysis of PRRT2 for benign infantile epilepsy,infantile convulsions with choleoathetosis syndrom, and benign convulsions with mild gastroenteritis

    • Author(s)
      Ishii A, Yasumoto S, Ihara Y, Inoue T, Fujita T, Nakamura N, Ohfu M, Lee WT, Kaneko S, Hirose S.
    • Organizer
      2012 Korea Epilepsy Congress
    • Place of Presentation
      Incheon, Korea
    • Related Report
      2012 Annual Research Report
  • [Presentation] Genetic analysis of Alternating Hemiplegia of Childhood

    • Author(s)
      Ishii A, Saito Y, Sasaki M, Hirose S.
    • Organizer
      10th European Congress of Epileptology
    • Place of Presentation
      London, UK
    • Related Report
      2012 Annual Research Report
  • [Book] Jesper's Basic Mechanisms of the Epilepsies2012

    • Author(s)
      Steinlein andOK, Kaneko S, HiroseS
    • Publisher
      Nicotinic acwtycholine receptor mutations
    • Related Report
      2012 Final Research Report
  • [Book] Nicotinic acetylcholine receptor mutations2012

    • Author(s)
      Steinlein OK, Kaneko S, Hirose S.
    • Publisher
      Jesper's Basic Mechanisms of the Epilepsies
    • Related Report
      2012 Annual Research Report
  • [Remarks]

    • URL

      http://www.med.fukuoka-u.ac.jp/epilepsy/

    • Related Report
      2012 Final Research Report

URL: 

Published: 2011-08-05   Modified: 2023-03-16  

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