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Trafficking abnormality in genetic arrhythmias and its modulation.

Research Project

Project/Area Number 16590664
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Circulatory organs internal medicine
Research InstitutionNiigata University

Principal Investigator

AIZAWA Yoshifusa  Niigata University, Institute of Medicine and Dentistry, Professor, 医歯学系, 教授 (50143780)

Co-Investigator(Kenkyū-buntansha) HANAWA Haruo  Niigata University, Institute of Medicine and Dentistry, Lecturer, 医歯学系, 講師 (40282983)
CHINUSHI Masaomi  Niigata University, Institute of Medicine and Dentistry, Associate Professor, 医歯学系, 助教授 (40303151)
YAMASHITA Fumio  Niigata University, Medical and Dental Hospital, Resident, 医歯学総合病院, 医員 (90401752)
鷲塚 隆  新潟大学, 大学院・医歯学総合研究科, 助手 (00301185)
Project Period (FY) 2004 – 2005
Project Status Completed (Fiscal Year 2005)
Budget Amount *help
¥3,100,000 (Direct Cost: ¥3,100,000)
Fiscal Year 2005: ¥1,200,000 (Direct Cost: ¥1,200,000)
Fiscal Year 2004: ¥1,900,000 (Direct Cost: ¥1,900,000)
Keywordsgenetic arrhythmias / channel protein / electrophysiology / trafficking / 遺伝子性不整脈
Research Abstract

1. Screening of Genes of myocardial channels and functional assay.
In congenital long QT syndrome, we found abnormal genes coding potassium channel (mainly LQT1) and 5 were de novo. Of these, the amino-acid at the filter site of the pore was found abnormal and loss of function was confirmed. The structure of the membrane domain of LQT1 by a computer showed precisely the abnormal site. New abnormal genes were also found in catecholaminergic polymorphic ventricular tachycardia and reported as the first case from Japan. Totally negative results for gene abnormalities were found in Brugada syndrome, so far.
2. Modulation of trafficking defect.
The C terminal is essential for trafficking of LQT1 and found two cases showing trafficking defect. One is with a case with T578M mutation but the prediction of structural change of the C-terminal of LQT1 by computer analysis was failed. To modulate the trafficking defect, RNA was attempted but mRNA with a single mutation was not suppressed. This led to the re-study of RNAi in mRNA with co-expressed with wild type. Over-expression of K-channel by transfection was possible but, so far, it provided a model of arrhythmia.

Report

(3 results)
  • 2005 Annual Research Report   Final Research Report Summary
  • 2004 Annual Research Report
  • Research Products

    (49 results)

All 2006 2005 2004 Other

All Journal Article (49 results)

  • [Journal Article] Over-expression of Kv1.5 in Rat Cardiomyocytes Extremely Shortens the Duration of the Action Potential and Causes Rapid Excitation2006

    • Author(s)
      Aizawa Y, et al.
    • Journal Title

      BBRC (印刷中)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Brugada Syndrome and Vasospasitc Angina Do Coexist.2006

    • Author(s)
      Aizawa Y, et al.
    • Journal Title

      Intern Medicine (印刷中)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Annual Research Report 2005 Final Research Report Summary
  • [Journal Article] Distinct U Wave Changes in Ptients Catecholaminergic Polymorphic Ventricular Tachycardia(CPVT)2006

    • Author(s)
      Aizawa Y, et al.
    • Journal Title

      International Heart Journal (印刷中)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Annual Research Report 2005 Final Research Report Summary
  • [Journal Article] Osborn Wave in Accidental Hypothermia2006

    • Author(s)
      Aizawa Y, et al.
    • Journal Title

      Internal Medicine 45

      Pages: 333-334

    • NAID

      130000076559

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Over-expression of Kv1.5 in Rat Cardiomyocytes Extremely Shortens the Duration of the Action Potential and Causes Rapid Excitation2006

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Biochem Biophys Rapid Comm (in press)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Distinct U Wave Changes in Ptients Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)2006

    • Author(s)
      Aizawa Y, et al.
    • Journal Title

      International Heart Journal (in press)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Human Cardiac RyR Mutations in lon Channel disorders in Japan2006

    • Author(s)
      Aizawa Y, et al.
    • Journal Title

      Heart (印刷中)

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Osborn Wave in Accidental Hypothermia2006

    • Author(s)
      Aizawa Y, et al.
    • Journal Title

      Internal Medicine (印刷中)

    • NAID

      130000076559

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Idiopathic ventricular fibrillation refractory to ICD therapy2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Internal Medicine 44

      Pages: 777-778

    • NAID

      130000076332

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Annual Research Report 2005 Final Research Report Summary
  • [Journal Article] A novel mutation in FKBP12.6 binding region of the human cardiac ryanodine receptor gene (R2401H) in a Japanese patient with catecholaminergic polymorphic ventricular tachycardia.2005

    • Author(s)
      Aizawa Y, et al.
    • Journal Title

      International Journal of Cardiology Mar18:99 (2)

      Pages: 343-345

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Effects of intravenous magnesium in a prolonged QT interval model of polymorphic ventricular tachycardia focus on transmural ventricular repolarization.2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Pacing and Clinical Electrophysiology 28

      Pages: 844-850

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Annual Research Report 2005 Final Research Report Summary
  • [Journal Article] Inarrpopriate discharges of intravenous cardioverteer defibrillators owing to lead failure.2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      International Heart Journal 46(5)

      Pages: 909-913

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Annual Research Report 2005 Final Research Report Summary
  • [Journal Article] Variable electrocardiographic effects of short-term quinidine sulfate administration in Brugada syndrome.2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Pacing and Clinical Electrophysiology May28(5)

      Pages: 372-377

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Annual Research Report 2005 Final Research Report Summary
  • [Journal Article] Genomic and non-genomic regulation of L-type calcium channels in rat ventricle by thyroid hormone.2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Endocrine Research 31(1)

      Pages: 59-70

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Annual Research Report 2005 Final Research Report Summary
  • [Journal Article] Electrophysiologic study-guided therapy with sotalol for life-threatening ventricular tachyarrhythmias.2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Pacing and Clinical Electrophysiology Apr28(4)

      Pages: 285-290

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Unsuccessful internal defibrillation in Brugada syndrome focus on refractoriness and ventricular fibrillation cycle length.2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Journal of Cardiovascular Electrophysiology Mar16(3)

      Pages: 562-566

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Idiopathic ventricular fibrillation refractory in ICD therapy.2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Internal Medicine 44

      Pages: 777-778

    • NAID

      130000076332

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] A novel mutation in FKBP12.6 binding region of the human cardiac ryanodine receptor gene (R2401H) in a Japanese patient with catecholaminergic polymorphic ventricular tachycardia.2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      International Journal of Cardiology 99

      Pages: 343-345

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Effects of intravenous magnesium in a prolonged QT interval model of polymorphic ventricular tachycardia focus on transmural ventricular repolarization.2005

    • Author(s)
      Chinushi M. et al.
    • Journal Title

      Pacing and Clinical Electrophysiology 28

      Pages: 844-850

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Inarrpopriate discharges of intravenous cardioverteer defibrillators owing to lead failure.2005

    • Author(s)
      Washizuka T et al.
    • Journal Title

      International Heart Journal 46

      Pages: 909-913

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Variable electrocardiographic effects of short-term quinidine sulfate administration in Brugada syndrome.2005

    • Author(s)
      Watanabe H. et al.
    • Journal Title

      Pacing and Clinical Electrophysiology 28

      Pages: 372-377

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Genomic and non-genomic regulation of L-type calcium channels in rat ventricle by thyroid hormone.2005

    • Author(s)
      Watanabe H. et al.
    • Journal Title

      Endocrine Research 31

      Pages: 59-70

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Electrophysiologic study-guided therapy with sotalol for life-threatening ventricular tachyarrhythmias.2005

    • Author(s)
      Watanabe H et al.
    • Journal Title

      Pacing and Clinical Electrophysiology

      Pages: 285-290

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Unsuccessful internal defibrillation in Brugada syndrome : focus on refractoriness and ventricular fibrillation cycle length.2005

    • Author(s)
      Watababe H, et al.
    • Journal Title

      Journal of Cardiovascular Electrophysiology 16

      Pages: 562-566

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] A novel mutation in FKBP12.6 binding region of the human cardiac ryanodine receptor gene (R2401H) in a Japanese patient with catecholaminergic polymorphic ventricular tachycardia.2005

    • Author(s)
      Aizawa Y, et al.
    • Journal Title

      International Journal of Cardiology Mar18:99(2)

      Pages: 343-345

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Electrophysiologic study-guided therapy with sotalol for life-threatening ventricular tachyarrhythmias.2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Pacing and Clininical Electrophysiology Apr28(4)

      Pages: 285-290

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Unsuccessful internal defibrillation in Brugada syndrome : focus on refractoriness and ventricular fibrillation cycle length.2005

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Journal of Cardiovascular Electrophysiology Mar16(3)

      Pages: 262-266

    • Related Report
      2005 Annual Research Report
  • [Journal Article] A novel mutastion in FKBP12.6 binding region of the human cardiac ryanodine receptor gene(R240H) in a Japanese patient with catechlaminergic polumorphic ventricular tachycardia.2005

    • Author(s)
      Aizawa Y, Ueda K, Komura, Aizawwa Y et al.
    • Journal Title

      International Journal of Cardiology 99

      Pages: 343-5

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Truncated KCNQ1 mutant, A178fs/105, forms hetero-multimer channel with wild-type causing a dominant-negative suppression due to trafficking defect.2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      FEBS Letter 574

      Pages: 145-150

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Indications for an implantable cardioverter defibrillator (ICD).2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Intern Medicine 43

      Pages: 360-390

    • NAID

      10013090822

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Catheter Ablation of Atrial Fibrillation : Its Indication and Problems.2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Internal Medicine 43

      Pages: 164-166

    • NAID

      130000084304

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Ventricular Tachyarrhythmia Associated with Cardiac Sarcoidosis : Its Mechanisms and Outcome.2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Clinical Cardiology 27

      Pages: 217-222

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Radiofrequency Catheter Ablation for Incessant Atrioventricular Nodal Reentrant Tachycardia Normalized H-V Block Associated With Tachycardia-induced Cardiomyopathy.2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Journal of Electrocardiology 37:4

      Pages: 315-319

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Linkage Between Mechanical and Electrical Alternans in Patients with Chronic Heart Failure.2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Journal of Cardiovascular Electrophysiology 15

      Pages: 295-299

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Potential Candidates for Carsiac Resynchronization Therapy in Japanese Patients With Idiopathic Dilated Cardiomyopathy -A Niigata Multicenter Study of DCM-2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Circulation Journal 68:12

      Pages: 1104-1109

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Mutation site-specific differences in arrhythmic risk and sensitivity to sympathetic stimulation in the LQT1 form of congentital long QT syndrome multicenter study in Japan.2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Journal of American College of Cardiology 44

      Pages: 117-125

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Truncated KCNQ1 mutant, A178fs/105 forms hetero-multimer channel with wild-type causing a dominant-negative suppression due to trafficking defect.2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      FEBS Letter 574

      Pages: 145-150

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Indications for an implantable cardioverter defibrillator (ICD).2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Internal Medicine. 43

      Pages: 360-370

    • NAID

      10013090822

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Ventricular Tachyarrhythmia Associated with Cardiac Sarcoidosis : Its Mechanisms and Outcome.2004

    • Author(s)
      Furushima H et al.
    • Journal Title

      Clinical Cardiology 27

      Pages: 217-222

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Radiofrequency Catheter Ablation for Incessant Atrioventricular Nodal Reentrant Tachycardia Normalized H-V Block Associated With Tachycardia-induced Cardiomyopathy.2004

    • Author(s)
      Furushima H et al.
    • Journal Title

      Journal of Electrocardiology 37

      Pages: 315-319

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Linkage Between Mechanical and Electrical Alternans in Patients with Chronic Heart Failure.2004

    • Author(s)
      Kodama M et al.
    • Journal Title

      Journal of Cardiovascular Electrophysiology 15

      Pages: 295-299

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Potential Candidates for Carsiac Resynchronization Therapy in Japanese Patients With Idiopathic Dilated Cardiomyopathy -A Niigata Multicenter Study of DCM-2004

    • Author(s)
      Komura S et al.
    • Journal Title

      Circulation Journal 68

      Pages: 1104-1109

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Mutation site-specific differences in arrhythmic risk and sensitivity to sympathetic stimulation in the LQT1 form of congentital long QT syndrome ; multicenter study in Japan.2004

    • Author(s)
      Shimizu W et al.
    • Journal Title

      Journal of American College of Cardiology 44

      Pages: 117-125

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Truncated KCNQ1 mutant, A178fs/105,forms hetero-multimer channel with wild-type causing a dominant-negative suppression due to trafficking defect.2004

    • Author(s)
      Aizawa Y, Hiraoka M, Aizawa Y, et al.
    • Journal Title

      FEBS Left 574(1-3)

      Pages: 145-50

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Mutation site-specific differences in arrhythmic risk and sensitivity to sympathetic stimulation in the LQT1 form of congentital long QT syndrome ; multicenter study in Japan.2004

    • Author(s)
      Shimizu W, Aizawa Y.et al.
    • Journal Title

      Journal of American College of Cardiology 44

      Pages: 117-125

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Postprandial Variations in ST-Segment in Patient with Brugada Syndrome and Partial Gastrectomy.2004

    • Author(s)
      Watanabe H, Aizawa Y et al.
    • Journal Title

      Pacing and Clinical Electrphisiology 27

      Pages: 1560-2

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Indications for an implantable cardioverter defibrillator(ICD).2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Internal Medicien 43

      Pages: 360-7

    • NAID

      10013090822

    • Related Report
      2004 Annual Research Report
  • [Journal Article] atheter Ablation of Atrial Fibrillation : Its Indication and Problems2004

    • Author(s)
      Aizawa Y et al.
    • Journal Title

      Internal Medicine 43

      Pages: 164-166

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Brugada Syndrome and Vasospasitc Angina Do Coexist.

    • Author(s)
      Aizawa Y et al.
    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary

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Published: 2004-04-01   Modified: 2016-04-21  

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