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2008 Fiscal Year Final Research Report

The cause and pathophysiology of common variable immunodeficiency

Research Project

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Project/Area Number 19591253
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionKyushu University

Principal Investigator

TAKADA Hidetoshi  Kyushu University, 大学病院, 特任准教授 (70294931)

Co-Investigator(Kenkyū-buntansha) TSUTSUMI Yasushi  九州大学, 大学病院, 助教 (80419564)
TANAKA Tamami  九州大学, 大学院医学研究院, 学術研究員 (60423547)
Project Period (FY) 2007 – 2008
Keywords原発性免疫不全症候群 / 分類不能型低ガンマグロブリン血症 / マイクロアレイ / 定量的PCR
Research Abstract

分類不能型低ガンマグロブリン血症患者末梢血単核球からCD4^+T 細胞を純化し、Th2細胞をin vitro で誘導し、Th2 細胞における遺伝子発現をマイクロアレイにてえ健常者と比較した。健常者と比較して、APOB、CHIM、FLIP1、SLC22A4、TMEM1、UPF2、FAH、SERPINA5、GGCX、SMARCAL1、遺伝子の発現量の低下が著しかった。これらの遺伝子の塩基配列を決定したが、遺伝子異常は認められなかった。次に患者末梢血B細胞を純化し、in vitroで活性化した後、遺伝子発現量を健常者と比較した。その結果、IGJ、ROR1、FLJ2、PDE3B、CNN3、SGCE、GNG2、TLE1 遺伝子の発現量の低下が著しかった。これらの遺伝子の塩基配列を決定したが、遺伝子異常は認められなかった。今後はさらにそれ以外の遺伝子で発現量の低下しているものに関して遺伝子解析を行い原因を明らかにしたい。

  • Research Products

    (35 results)

All 2009 2008 2007

All Journal Article (25 results) Presentation (10 results)

  • [Journal Article] FBP17 Mediates a Common Molecular Step in the Formation of Podosomes and Phagocytic Cups in Macrophages2009

    • Author(s)
      Tsuboi S, Takada H, Hara T, Mochizuki N, Funyu T, Saitoh H, Terayama Y, Yamaya K, Ohyama C, Nonoyama S, Ochs HD.
    • Journal Title

      J Biol Chem 284

      Pages: 8548-56

  • [Journal Article] Cardiovascular Complications Associated with Chronic Active Epstein-Barr Virus Infection2009

    • Author(s)
      Muneuchi J, Ohga S, Ishimura M, Ikeda K, Yamaguchi K, Nomura A, Takada H, Abe Y, Hara T.
    • Journal Title

      Pediatr Cardiol (Epub ahead of print)

  • [Journal Article] Lipopolysaccharide-induced monocytic cell death for the diagnosis of mild neonatal-onset multisystem inflammatory disease2008

    • Author(s)
      Takada H, Ishimura M, Inada H, Ohga S, Kusuhara K, Moroi Y, Furue M, Hara T.
    • Journal Title

      J Pediatr 152

      Pages: 885-7

  • [Journal Article] Epstein-Barr virus load in cerebrospinal fluid of patients with chronic active Epstein-Barr virus infection2008

    • Author(s)
      Ohga S, Sanefuji M, Ishimura M, Nomura A, Torisu H, Kira R, Takada H, Mizuno Y, Kazuyama Y, Hara T.
    • Journal Title

      Pediatr Infect Dis J 27

      Pages: 1027-30

  • [Journal Article] Sjogren's syndrome-associated meningoencephalomyelitis : cerebrospinal fluid cytokine levels and therapeutic utility of tacrolimus2008

    • Author(s)
      Hoshina T, Yamaguchi Y, Ohga S, Kira R, Ishimura M, Takada H, Tanaka T, Hara T.
    • Journal Title

      J Neurol Sci 267

      Pages: 182-6

  • [Journal Article] Thromboembolic complications in splenectomized patients with dominantly inherited beta-thalassemia2008

    • Author(s)
      Ohga S, Ideguchi H, Kato J, Ishimura M, Takada H, Harada N, Kawanaka H, Hattori Y, Kang D, Hamasaki N, Hara T.
    • Journal Title

      Acta Haematol 120

      Pages: 31-5

  • [Journal Article] Epstein-Barr virus-associated meningoencephalomyelitis : intrathecal reactivation of the virus in an immunocompetent child2008

    • Author(s)
      Sanefuji M, Ohga S, Kira R, Nomura A, Torisu H, Takada H, Kusuhara K, Hara T.
    • Journal Title

      J Child Neurol 23

      Pages: 1072-7

  • [Journal Article] 血球貪食症候群の発症機序2008

    • Author(s)
      高田英俊、大賀正一、原寿郎
    • Journal Title

      サイトカイン血液・腫瘍科 57

      Pages: 40-5

  • [Journal Article] EBウイルスに伴う血球貪食症候群2008

    • Author(s)
      大賀正一、石村匡崇、菅尚浩、野村明彦、高田英俊、原寿郎
    • Journal Title

      発症機序血液・腫瘍科 57[Suppl.6]

      Pages: 116-21

  • [Journal Article] 自然免疫による免疫不全症2008

    • Author(s)
      高田英俊、原寿郎
    • Journal Title

      小児科臨床 61

      Pages: 1783-9

  • [Journal Article] インターフェロンガンマ受容体異常症と類似疾患2008

    • Author(s)
      高田英俊、原寿郎
    • Journal Title

      小児内科40増刊号小児疾患診療のための病態生理 1

      Pages: 1356-9

  • [Journal Article] Disseminated Bacillus Calmette-Guerin lymphadenitis in a patient with gp91phox (-) chronic granulomatous disease 25 years after vaccination2008

    • Author(s)
      Kusuhara K, Ohga S, Hoshina T, Saito M, Sasaki Y, Ishimura M, Takada H, Fujita M, Hara T.
    • Journal Title

      Eur J Pediatr (Epub ahead of print)

  • [Journal Article] Fulminant sepsis/meningitis due to Haemophilus influenzae in a protein C-deficient heterozygote treated with activated protein C therapy2008

    • Author(s)
      Ishimura M, Saito M, Ohga S, Hoshina T, Baba H, Urata M, Kira R, Takada H, Kusuhara K, Kang D, Hara T.
    • Journal Title

      Eur J Pediatr (Epub ahead of print)

  • [Journal Article] Lack of association between E148Q MEFV variant and Kawasaki disease2008

    • Author(s)
      Yamaguchi K, Ikeda K, Ihara K, Takada H, Kusuhara K, Hara T
    • Journal Title

      Hum Immunol (Epub ahead of print)

  • [Journal Article] Tissue inhibitor of metalloproteinase 2 and coronary artery lesions in Kawasaki disease2007

    • Author(s)
      Furuno K, Takada H, Yamamoto K, Ikeda K, Ohno T, Khajoee V, Mizuno Y, Hara T
    • Journal Title

      J Pediatr 151

      Pages: 155-60

  • [Journal Article] Dominant-negative mutations in the DNA-binding domain of STAT3 cause hyper-IgE syndrome2007

    • Author(s)
      Minegishi Y, Saito M, Tsuchiya S, Tsuge I, Takada H, Hara T, Kawamura N, Ariga T, Pasic S, Stojkovic O, Metin A, Karasuyama H.
    • Journal Title

      Nature 448

      Pages: 1058-62

  • [Journal Article] Selective predisposition to bacterial infections in IRAK-4-deficient children: IRAK-4-dependent TLRs are otherwise redundant in protective immunity2007

    • Author(s)
      Ku CL, von Bernuth H, Picard C, Zhang SY, Chang HH, Yang K, Chrabieh M, Issekutz AC, Cunningham CK, Gallin J, Holland SM, Roifman C, Ehl S, Smart J, Tang M, Barrat FJ, Levy O, McDonald D, Day-Good NK, Miller R, Takada H, Hara T, Al-Hajjar S, Al-Ghonaium A, Speert D, Sanlaville D, Li X, Geissmann F, Vivier E, Marodi L, Garty BZ, Chapel H, Rodriguez-Gallego C, Bossuyt X, Abel L, Puel A, Casanova JL.
    • Journal Title

      J Exp Med 204

      Pages: 2407-22

  • [Journal Article] 本邦初のInterleukin-1 receptor associated kinase 4欠損症兄弟例の臨床的特徴2007

    • Author(s)
      吉川秀人、北村太郎、渡辺周永、虻川大樹、稲垣徹史、武山淳二、今泉益栄、高田英俊、原寿郎
    • Journal Title

      日本小児科学会雑誌 111

      Pages: 750-4

  • [Journal Article] Successful self-infusion of activated prothrombin complex concentrate for prophylaxis in a child with a factor VIII inhibitor2007

    • Author(s)
      Ohga S, Nomura A, Takada H, Suga N, Hara T.
    • Journal Title

      Am J Hematol 82

      Pages: 145-9

  • [Journal Article] Early-onset sarcoidosis mimicking refractory cutaneous histiocytosis2007

    • Author(s)
      Ohga S, Ichino K, Urabe K, Ishimura M, Takada H, Nishikomori R, Furue M, Hara T.
    • Journal Title

      Pediatr Blood Cancer 50

      Pages: 723-6

  • [Journal Article] Malignant hepatic tumor occurring 10 yrs after a histocompatible sibling donor bone marrow transplantation for severe aplastic anemia2007

    • Author(s)
      Ishimura M, Ohga S, Nagatoshi Y, Okamura J, Tajiri T, Kohashi K, Oda Y, Takada H, Hara T.
    • Journal Title

      Pediatr Transplant 11

      Pages: 945-9

  • [Journal Article] 結核菌/非定型抗酸菌に対する易感染性とIL-12/IFN-・経路2007

    • Author(s)
      楠原浩一、高田英俊、原寿郎
    • Journal Title

      日本サルコイドーシス/肉芽腫性疾患学会雑誌 27

      Pages: 9-19

  • [Journal Article] 血球貪食症候群2007

    • Author(s)
      大賀正一、野村明彦、高田英俊、原寿郎
    • Journal Title

      小児内科 39

      Pages: 2078-81

  • [Journal Article] 慢性活動性EBウイルス感染症-病態と治療-2007

    • Author(s)
      大賀正一、石村匡崇、野村明彦、高田英俊、原寿郎
    • Journal Title

      日本小児皮膚科学会雑誌 26

      Pages: 69-73

  • [Journal Article] 自己炎症性症候群2007

    • Author(s)
      原寿郎、高田英俊、楠原浩一
    • Journal Title

      小児科臨床 60

      Pages: 1505-16

  • [Presentation] Novel primary immunodeficiency syndrome with a developmental defect of pro-B cells2008

    • Author(s)
      Hidetoshi Takada, Yojiro Arinobu, Masataka Ishimura, Koichi Akashi, Toshiro Hara
    • Organizer
      NK cells and plasmacytoid dendritic cells第38回日本免疫学会
    • Place of Presentation
      京都
    • Year and Date
      20081201-03
  • [Presentation] Development of functional human B cells in NOD/SCID/IL2r ・KO mice and the application of the xenotransplant model in studying human immunodeficiency2008

    • Author(s)
      Takehiko Doi, TAKADA Hidetoshi, KANEGANE Hirokazu, TOMIZAWA Mariko, NAKAYAMA Toshinori, OHARA Osamu, MIYAWAKI Toshio, HARA Toshiro, ISHIKAWA Fumihiko
    • Organizer
      第38回日本免疫学会総会・学術集会
    • Place of Presentation
      京都
    • Year and Date
      20081201-03
  • [Presentation] Pathophysiology and treatment of Diamond-Blackfan anemia SYMPOSIUM 4 Hematological disorders in mature and premature infants by JSPS-NRCT program and the 18th Annual Meeting of the Japan Society of Obstetrical2008

    • Author(s)
      Ohga S, Koga Y, Ishimura M, Takada H, Hara T
    • Organizer
      Gynecological and Neonatal Hematology
    • Place of Presentation
      Fukuoka
    • Year and Date
      20080727-28
  • [Presentation] NEMO mutation as a cause of familial occurrence of female Behcet's disease patisnts2008

    • Author(s)
      Hara T, Takada H
    • Organizer
      XX International Congress of Genetics
    • Place of Presentation
      Berlin, Germany
    • Year and Date
      20080716-17
  • [Presentation] Central nervous system reactivation of Epstein-Barr virus (EBV) in chronic active EBV infection and EBV-associated encephalitis The 3rd Congress of Asian Society for Pediatric Research2007

    • Author(s)
      Ohga S, Sanefuji M, Ishimura M, Nomura A, Torisu H, Kira R, Takada H, Mizuno Y, Kazuyama Y, Hara T
    • Place of Presentation
      Tokyo, Japan
    • Year and Date
      20071007-08
  • [Presentation] Clinical outcomes of cardiac complications associated with chronic active Epstein-Barr virus infection (CAEBV)2007

    • Author(s)
      Muneuchi J, Ohga S, Ishimura M, Yamawaki K, Kanaya Y, Takada H, Hara T
    • Organizer
      The 3rd Congress of Asian Society for Pediatric Research (Oral)
    • Place of Presentation
      Tokyo, Japan
    • Year and Date
      20071007-08
  • [Presentation] Malignant hepatic tumor occurring 10 years after a histocompatible sibling donor bone marrow transplantation for severe aplastic anemia2007

    • Author(s)
      Ishimura M, Ohga S, Nagatoshi Y, Okamura J, Tajiri T, Kohashi K, Oda Y, Takada H, Hara T
    • Organizer
      The 3rd Congress of Asian Society for Pediatric Research
    • Place of Presentation
      Tokyo, Japan
    • Year and Date
      20071007-08
  • [Presentation] Genetic backgrounds of patients with susceptibility to mycobacterial infections in Japan2007

    • Author(s)
      Hara T, Takada H, Hoshina T, Sasaki Y, Saito M, Nomura A, Kusuhara K, Kobayashi M, Miyawaki T
    • Organizer
      25th International Congress of Pediatrics
    • Place of Presentation
      Athens, Greece
    • Year and Date
      20070825-30
  • [Presentation] Malignant hepatic tumor occurring 10 years after a histocompatible sibling donor bone marrow transplantation for severe aplastic anemia 14th Annual Meeting of the Japan Childhood2007

    • Author(s)
      Ishimura M, Ohga S, Nagatoshi Y, Okamura J, Oda Y, Takada H, Hara T
    • Organizer
      Aplastic Anemia Study Group
    • Place of Presentation
      Nagoya, Japan
    • Year and Date
      20070602-03
  • [Presentation] Congenital pure red cell aplasia as a human ribosomopathy SESSION I2007

    • Author(s)
      Ohga S, Koga Y, Ishimura M, Nomura A, Takada H, Hara T
    • Organizer
      Prenatal and Pediatric Research for Future Development of Cure, Care and Prevention of Congenital Disease The 18th Fukuoka International Symposium on Pediatric/Maternal-Child Health Research
    • Place of Presentation
      Fukuoka,Japan
    • Year and Date
      2007-09-01

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Published: 2010-06-10   Modified: 2016-04-21  

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