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2012 Fiscal Year Final Research Report

Application of humanized mice to analyses and therapeutics of phagocytic disorders

Research Project

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Project/Area Number 22591161
Research Category

Grant-in-Aid for Scientific Research (C)

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

Principal Investigator

KOBAYASHI Masao  広島大学, 大学院・医歯薬保健学研究院, 教授 (00162016)

Co-Investigator(Kenkyū-buntansha) KAJIUME Teruyuki  広島大学, 大学院・医歯薬保健学研究院, 助教 (40278924)
Project Period (FY) 2010 – 2012
Keywords小児血液学 / ヒト化マウス
Research Abstract

The use of genetically modified mice has led to significant advantages for the examination of in disease pathophysiology. In this study, CD34 cells purified from bone marrow in patients with severe congenital neutropenia were injected to NOD/SCID/・cnull(NOG) mice intravenously. The human cells engrafted in mice showed the maturation arrest of myeloid cells as well as the absence of mature human neutrophils. These results suggest that the establishment of humanized mice may significantly contribute to analyses of pathophysiology and therapeutic approach in patients with SCN.

  • Research Products

    (35 results)

All 2013 2012 2011 2010

All Journal Article (19 results) (of which Peer Reviewed: 17 results) Presentation (11 results) Book (5 results)

  • [Journal Article] Identification of the integrin b3 L718P mutation in a pedigree with autosomal dominant thrombocytopenia with anisocytosis2013

    • Author(s)
      Kobayashi Y, Matsui H, Kanai A, Tsumura M, Okada S, Miki M, Nakamura K, Kunishima S, Inaba T, Kobayashi M
    • Journal Title

      British Journal of Haematology

      Volume: 160 Pages: 521-529

    • Peer Reviewed
  • [Journal Article] Wnt3a stimulates maturation of impaired neutrophils developed from severe congenital neutropenia-derived pluipotent stem cells with heterozygous ELANEmutation2013

    • Author(s)
      Hiramoto T, Ebihara Y, Mizoguchi Y, Nakamura K, Yamaguchi K, Ueno K, Mochizuki S, Yamamoto S, Nagasaki M, Furukawa Y, Tani K, Nakauchi H, Kobayashi M, Tsuji K
    • Journal Title

      Proc Natl Acad Sci USA

      Volume: 110 Pages: 3023-8

    • Peer Reviewed
  • [Journal Article] Frequent somatic mosaicism of NEMO in T cells of patients with X-linked anhidrotic ectodermal dysplasia and immunodeficiency2012

    • Author(s)
      Kawai T, Nishikomori R, Izawa K, Murata Y, Tanaka N, Sakai H, Saito M, Yasumi T, Takaoka Y, Nakahata T, Mizukami T, Nunoi H, Kiyohara Y, Yoden A, Murata T, Sasaki S, Ito E, Akutagawa H, Kawai T, Imai C, Okada S, Kobayashi M, Heike T
    • Journal Title

      Blood

      Volume: 119 Pages: 5458-5466

    • Peer Reviewed
  • [Journal Article] Dominant-negative STAT1 SH2 domain mutations in unrelated patients with Mendelian susceptibility to mycobacterial disease2012

    • Author(s)
      Tsumura M, Okada S, Sakai H, Yasunaga S, Ohtsubo M, Murata T, Obata H, Yasumi T, Kong XF, Abhyankar A, Heike T, Nakahata T, Nishikomori R, Al-Muhsen S, Boisson-Dupuis S, Casanova JL, Alzahrani M, Shehri MA, Elghazali G, Takihara Y, Kobayashi M
    • Journal Title

      Human Mutation

      Volume: 33 Pages: 1377-1387

    • Peer Reviewed
  • [Journal Article] Oncogenic fusion E2A-HLF sensitizes t(17:19)-positive acute lymphoblastic leukemia to TRAIL-mediated apoptosis by upregulating the expression of death receptors2012

    • Author(s)
      Zhang X, Inukai T, Hirose K, Akahane K, Kuroda I, Honna-Oshiro H, Kagami K, Goi K, Nakamura K, Kobayashi M, Endo M, Yagita H, Kurosawa H, Look TA, Honda H, Inaba T, Nakazawa S, Sugita K
    • Journal Title

      Leukemia

      Volume: 26 Pages: 2843-93

    • Peer Reviewed
  • [Journal Article] Transmission of a maternal EDTA-dependent anticoagulant.2012

    • Author(s)
      Ohno N, Kobayashi M, Hayakawa S, Utsunomiya A, Karakawa
    • Journal Title

      Platelets

      Volume: 23 Pages: 399-400

    • Peer Reviewed
  • [Journal Article] Regulation of hematopoietic stem cells using transduction domain-fused polycomb2012

    • Author(s)
      Kajiume T, Sera Y, Kawahara Y, Matsumoto M, Fukazawa T, Imura T, Yuge L, Kobayashi M
    • Journal Title

      Experimantal Hematology

      Volume: 40 Pages: 751-760

    • Peer Reviewed
  • [Journal Article] Glanzmann thrombasthenia (GT) の遺伝子組み換え活性型第Glanzmann thrombasthenia (GT) の遺伝子組み換え活性型第VII因子製剤による止血効果因子製剤による止血効果2012

    • Author(s)
      溝口洋子,鎌田綾,三木瑞香,谷博雄,世羅康彦,中村和洋,小林正夫
    • Journal Title

      日本小児血液・がん学会雑誌

      Volume: 49 Pages: 61-66

    • Peer Reviewed
  • [Journal Article] 輸血後蕁麻疹発症前の末梢血一般検査所見2012

    • Author(s)
      梶梅輝之,浅野孝基,世羅康彦,小林正夫
    • Journal Title

      アレルギー

      Volume: 61 Pages: 1086-1091

    • Peer Reviewed
  • [Journal Article] 慢性皮膚粘膜カンジダ症と機能獲得性STAT1 変異2012

    • Author(s)
      溝口洋子,津村弥来,岡田 賢,小林正夫
    • Journal Title

      臨床免疫・アレルギー科

      Volume: 57 Pages: 437-443

  • [Journal Article] Decreased Expression in Nuclear Factor-κB Essential Modulator Due to a Novel Splice-Site Mutation Causes X-linked Ectodermal Dysplasia with Immunodeficiency2011

    • Author(s)
      Karakawa S, Okada S, Tsumura M, Mizoguchi Y, Ohno N, Yasunaga S, Ohtsubo M, Kawai T, Nishikomori R, Sakaguchi T, Takihara Y, Kobayashi M
    • Journal Title

      Journal of Clinical Immunology

      Volume: 31 Pages: 762-77

    • Peer Reviewed
  • [Journal Article] Gain of function of human STAT1 mutations impair IL17 immunity and underlie chronic mucocutaneous candidiasis2011

    • Author(s)
      Liu L, Okada S, Kong XF, Kreins AY, Cypowyj S, Abhyankar A, Toubiana J, Itan Y, Audry M, Nitschke P, Masson C, Toth B, Flatot J, Migaud M, Chrabieh M, Kochetkov T, Bolze A, Borghesi A, Toulon A, Hiller J, Eyerich S, Eyerich K, Gulacsy V, Chernyshova L, Chernyshov V, Bondarenko A, Maria Cortes Grimaldo R, Blancas-Galicia L, Madrigal Beas IM, Roesler J, Magdorf K, Engelhard D, Thumerelle C, Burgel PR, Hoernes M, Drexel B, Seger R, Kusuma T, Jansson AF, Sawalle-Belohradsky J, Belohradsky B, Jouanguy E, Bustamante J, Bue M, Karin N, Wildbaum G, Bodemer C, Lortholary O, Fischer A, Blanche S, Al-Muhsen S, Reichenbach J, Kobayashi M, Rosales FE, Lozano CT, Kilic SS, Oleastro M, Etzioni A, Traidl-Hoffmann C, Renner ED, Abel L, Picard C, Marodi L, Boisson-Dupuis S, Puel A, Casanova JL
    • Journal Title

      Journal of Experimental Medicine

      Volume: 208 Pages: 1635-48

    • Peer Reviewed
  • [Journal Article] Clinical and host genetic characteristics of Mendelian susceptibility to mycobacterial diseases in Japan2011

    • Author(s)
      Hoshina T, Takada H, Sasaki-Mihara Y, Kusuhara K, Ohshima K, Okada S, Kobayashi M, Ohara O, Hara T
    • Journal Title

      Journal of Clinical Immunology

      Volume: 31 Pages: 309-14

    • Peer Reviewed
  • [Journal Article] Quantification of κ-deleting recombination excision circles in Guthrie cards for the identification of early B-cell maturation defects2011

    • Author(s)
      Nakagawa N, Imai K, Kanegane H, Sato H, Yamada M, Kondoh K, Okada S, Kobayashi M, Agematsu K, Takada H, Mitsuiki N, Oshima K, Ohara O, Suri D, Rawat A, Singh S, Pan-Hammarstrom Q, Hammarstrom L, Reichenbach J, Seger R, Ariga T, Hara T, Miyawaki T, Nonoyama S
    • Journal Title

      Journal of Allergy & Clinical Immunology

      Volume: 128 Pages: 223-225

    • Peer Reviewed
  • [Journal Article] Hoxb4 transduction down-regulates Geminin protein, providing hematopoietic stem and progenitor cells with proliferation potential2010

    • Author(s)
      Ohno Y, Yasunaga S, Ohtsubo M, Ori S, Tsumura M, Okada S, Ohta T, Kiyoshi Ohtani, Kobayashi M, Takihara Y
    • Journal Title

      Proc. Natl. Acad. Sci

      Volume: 107(50) Pages: 21529-34

    • Peer Reviewed
  • [Journal Article] Molecular pathogenesis of a novel mutation, G108D, in short-chain acyl-CoA dehydrogenase identified in subjects with short-chain acyl-CoA dehydrogenase deficiency2010

    • Author(s)
      Shirao K, Okada S, Tajima G, Tsumura M, Hara K, Yasunaga S, Ohtsubo M, Hata I, Sakura N, Shigematsu Y, Takihara Y, Kobayashi M
    • Journal Title

      Human Genetics

      Volume: 127 Pages: 619-28

    • Peer Reviewed
  • [Journal Article] Significance of immature platelet fraction and CD-41-positive cells at birth in early onset neonatal thrombocytopenia2010

    • Author(s)
      Kihara H, Ohno N, Karakawa W, Mizoguchi Y, Fukuhara R, Hayashidani M, Nomura S, Nakamura K, Kobayashi M
    • Journal Title

      International Journal of Hematology

      Volume: 91 Pages: 245-251

    • Peer Reviewed
  • [Journal Article] Hematological malignancies in congenital neutropenia2010

    • Author(s)
      Okada S, Nakamura K, Kobayashi M
    • Journal Title

      Rinsho Ketsueki

      Volume: 51 Pages: 553-558

    • Peer Reviewed
  • [Journal Article] 血液疾患における病態解析研究の進歩:先天性好中球減少症発症機構解明の進展2010

    • Author(s)
      溝口洋子,岡田 賢,小林正夫
    • Journal Title

      血液・腫瘍科

      Volume: 60(2) Pages: 118-124

  • [Presentation] Identification of integrin b3 L718P mutation in a pedigree with autosomal dominant macrothrombocytopenia2012

    • Author(s)
      Yoshiyuki Kobayashi, Hirotaka Matsui, Akinori Kanai, Miyuki Tsumura, Satoshi Okada, Mizuka Miki, Kazuhiro Nakamura, Shinji Kunishima, Toshiya Inaba and Masao Kobayashi
    • Organizer
      American Society of Hematology
    • Place of Presentation
      Atlanta, GA
    • Year and Date
      20121208-11
  • [Presentation] A Nobel Method using Extracted Human Neutrophil Antigens from HNA gene-transfected cell lines for Detection of Antibodies against Human Neutrophil Antigens2012

    • Author(s)
      Rie Onodera, Kazuhiro Nakamura, Kikuyo Taniguchi, Emi Kurita, Asako Hiraoka, Kaduta Yasui, Nobuki Matsuyama, Fumiya Hirayama, and Masao Kobayashi
    • Organizer
      American Society of Hematology
    • Place of Presentation
      Atlanta, GA
    • Year and Date
      20121208-11
  • [Presentation] Gain-of-function mutations of STAT1 in Japanese patients with CMCD2012

    • Author(s)
      Osamu Hirata, Miyuki Tsumura, Yoko Mizoguchi, Satoshi Okada, Shizuko Minegishi, Tomohiro Morio, Masao Kobayashi
    • Organizer
      European Society for Immunodeficiency
    • Place of Presentation
      Florence, Italy
    • Year and Date
      20121007-10
  • [Presentation] Clinical and genetic characteristics of patients with severe congenital neutropenia in Japan2011

    • Author(s)
      Mizoguchi Y, Nakamura K, Karakawa S, Okada S, Kawaguchi H, Kobayashi M
    • Organizer
      53rd American Society of Hematology Annual Meeting
    • Year and Date
      20111210-13
  • [Presentation] Suppressed Neutrophil Development in Hematopoiesis of Induced Pluripotent Stem Cells Derived From a Severe Congenital Neutropenia Patient with ELA2 Mutation2011

    • Author(s)
      Hiramoto T, Ebihara Y, Mizoguchi Y, Nakamura K, Mochizuki S, Yamamoto S, Matsuzaka E, Hanada S, Ohnishi R, Tani K, Eto K, Nakauchi H, Kobayashi M, Tsuji K
    • Organizer
      53rdAmerican Societey of Hematology Annual Meeting
    • Place of Presentation
      San Diego, CA
    • Year and Date
      20111210-13
  • [Presentation] JAK2 mutations and CRLF2 rearrangements in Down Syndrome-Associated Acute Lymphoblastic Leukemia in Japan2011

    • Author(s)
      Hanada I, Terui K, Toki T,Kudo K, Sato T, Kamio T, Sasaki S,Takahashi Y, Hayashi Y, Sugita K, Kojima S, Koike K, Kosaka Y, Kobayashi M, Ito E
    • Organizer
      53rdAmerican Societey of Hematology Annual Meeting
    • Place of Presentation
      San Diego, CA
    • Year and Date
      20111210-13
  • [Presentation] TsumuraM, Okada S, Sakai H, Nishikomori R, YasunagaS, Ohtsubo M, HeikeT, NakahataT, TakiharaY, KobayashiM2011

    • Author(s)
      TsumuraM, Okada S, Sakai H, Nishikomori R, YasunagaS, Ohtsubo M, HeikeT, NakahataT, TakiharaY, KobayashiM
    • Organizer
      第73回日本血液学会学術集会
    • Place of Presentation
      名古屋
    • Year and Date
      20111014-16
  • [Presentation] 常染色体優性遺伝を呈する慢性皮膚粘膜カンジダ症の責任遺伝子の発見-STAT1 機能獲得性変異による慢性皮膚粘膜カンジダ症-2011

    • Author(s)
      岡田 賢,小林正夫,Luyan Liu, Xiao-Fei Kong, Alexandra Y. Kreins, Sophie Cypowyj, Laurent Abel, Capucine Picard, Stephanie Boisson-Dupuis, Anne Puel, Jean-Laurent Casanova
    • Organizer
      第39回日本臨床免疫学会総会
    • Place of Presentation
      東京
    • Year and Date
      20110915-17
  • [Presentation] Umbilical cord blood-derived stem cells cultured under low oxygen tension enhance the migratioin and homing efficacy through the increased expression of CXCR42010

    • Author(s)
      Ohno N, Kajiume T, Hayakawa S, Kobayashi Y, Ytsunomiya A, Kobayashi M
    • Organizer
      52nd American Society of Hematology Annual Meeting
    • Place of Presentation
      Orlando FL USA
    • Year and Date
      20101204-07
  • [Presentation] A Novel mutation K673R in STAT1 impaired the STAT1 signal transduction in a dominant-negative manner identified in a Japanese boy2010

    • Author(s)
      Mizoguchi Y, Tsumura M, Okada S, Sakai H, Nishikomori R, Yasunaga S, Ohtsubo M, Murata T, Obata H, Yasumi T, Heike T, Nakahata T, Takaihara Y, Kobayashi M
    • Organizer
      52nd American Society of Hematology Annual Meeting
    • Place of Presentation
      Orlando FL USA
    • Year and Date
      20101204-07
  • [Presentation] Decreased expression in NF-kB essential modulator due to a novel splice-site mutation causes ectodermal dysplasia with immunodeficiency2010

    • Author(s)
      Karakawa S, Okada, S, Tsumura M, Yasunaga S, Ohtsubo M, Kawai T, Nishikomori R, Takihara Y, Kobayashi M
    • Organizer
      52nd American Society of Hematology Annual Meeting
    • Place of Presentation
      Orlando FL USA
    • Year and Date
      20101204-07
  • [Book] 免疫不全症:貪食細胞の数・機能の異常2012

    • Author(s)
      原 寿郎
    • Total Pages
      178-184
    • Publisher
      診断と治療社
  • [Book] 出血傾向2011

    • Author(s)
      佐地勉,竹内義博,原寿郎
    • Total Pages
      161-162
    • Publisher
      中外医学社
  • [Book] 出血性疾患2011

    • Author(s)
      佐地勉,竹内義博,原寿郎
    • Total Pages
      412-415
    • Publisher
      中外医学社
  • [Book] 好中球減少症2011

    • Author(s)
      小林正夫
    • Total Pages
      515-516
    • Publisher
      医学書院
  • [Book] Annual Review 20102010

    • Author(s)
      岡田賢,小林正夫
    • Total Pages
      41-49
    • Publisher
      中外医学社

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Published: 2014-08-29  

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