Pathological characterization of inflammatory bowel disease based on primary immunodeficiency

• Project/Area Number: 20K08718
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 54010:Hematology and medical oncology-related
• Research Institution: Wakayama Medical University
• Principal Investigator: 田村 志宣 和歌山県立医科大学, 医学部, 准教授 (10364085)
• Project Period (FY): 2020-04-01 – 2024-03-31
• Project Status: Completed (Fiscal Year 2023)
• Budget Amount: ¥3,770,000 (Direct Cost: ¥2,900,000、Indirect Cost: ¥870,000); Fiscal Year 2022: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000); Fiscal Year 2021: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000); Fiscal Year 2020: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
• Keywords: 原発性免疫不全症 / 炎症性腸疾患 / DNAリガーゼIV / 遺伝子再構成障害 / LIG4症候群 / Th1細胞 / IFN-γ / TCRレパトア解析 / ヘルパーT細胞 / DNAリガーゼIV（LIG4） / CD4陽性T細胞 / IFNγ

Outline of Research at the Start

原発性免疫不全症は, 免疫応答に関わる分子の遺伝子変異によって免疫制御機構が生まれつき破綻する疾患群であり, 易感染性を主徴とするが, 時に免疫機能の異常な活性化に伴う多彩な自己免疫病態を合併する. 我々は, 自験の原発性免疫不全症例において, DNA二本鎖切断修復に関わる酵素(DNAリガーゼIV)のhypomorphic mutationを同定し, その変異を導入したマウスが, 免疫不全を基盤として炎症性腸疾患を呈することを見出した. 本研究では, 申請者が独自に作成した変異マウスを用いて, 免疫機能が低下した状況下で, 臓器特異的な自己免疫病態がどのような機序で生じるのかを検討する.

Outline of Annual Research Achievements

本研究では、DNA損傷修復に関わるDNAリガーゼIV（LIG4）の低形成性変異を導入したマウス（Lig4W447Cホモ変異マウス）に生じた炎症性腸疾患の分子基盤、細胞生物学的基盤を明らかにすることを目的とした。これまでに、Lig4W447Cホモ変異マウスにおいて、DNA損傷修復障害、獲得免疫不全が生じること、さらには、IFN-γを産生するTh1細胞を主体とする炎症性腸疾患を発症することを見出した。今年度は、このホモ変異マウスに生じたユニークな炎症病態の解析を継続し、いくつかの結果を得ることができた。

１）Lig4W447Cホモ変異マウスとRag2欠損マウス（リンパ球欠失マウス）とのダブル変異マウスを作成した。このダブル変異マウスでは、完全に炎症を消去でき、長期生存した。Lig4W447Cホモ変異マウスの腸炎はリンパ球依存性であることを見出した。
２）Lig4W447Cホモ変異マウスの腸炎発症には、IFN-γが鍵となるサイトカインと考えられた。そこで、IFN-γ欠損マウスを作成し、Lig4W447Cホモ変異マウスとのダブル変異マウスを作成した。このダブル変異マウスでは、Lig4W447Cホモ変異マウスと同じく、大腸を含めた全身性の炎症が認められた。一方、FACS解析やRNA-Seq解析より、この炎症はTh17細胞がドミナントな表現型であった。IFN-γを欠損させることで、炎症病態がTh1型からTh17型に移行することを見出した。
３）野生型マウスとLig4W447Cホモ変異マウスの脾臓で、シングルセル解析とTCRレパトア解析を行った。シングルセル解析より、変異マウスでは、IFN-γを産生するTh1細胞がドミナントであることを見出し。さらに、TCRレパトア解析より、ホモ変異マウスでは、TCR遺伝子のclonotypeの著明な減少とoligoclonalなTCRの候補を見出した。

Research Products

• [Journal Article] Predictive Model for Occurrence of Febrile Neutropenia after Chemotherapy in Patients with Diffuse Large B-Cell Lymphoma: A Multicenter, Retrospective, Observational Study (2024)
  • Author(s): Morimoto Masaya、Yokoya Yuma、Yoshida Kikuaki、Kosako Hideki、Hori Yoshikazu、Mushino Toshiki、Tamura Shinobu、Ito Reiko、Koyamada Ryosuke、Yamashita Takuya、Mori Shinichiro、Mori Nobuyoshi、Ohde Sachiko
  • Journal Title: Hematology Reports Volume: 16 Issue: 1 Pages: 76-88
  • DOI: 10.3390/hematolrep16010008
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Post-infarction Left Ventricular Free Wall Rupture Diagnosed by Contrast-Enhanced Computed Tomography: A Case Report (2024)
  • Author(s): Morimoto Yuya、Tamura Shinobu、Kawashima Shuji、Inoue Shigeaki
  • Journal Title: Cureus Volume: 16
  • DOI: 10.7759/cureus.52127
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Clinicopathological analysis of CD47 and signal regulatory protein alpha expression in myeloid sarcoma patients: CD47 expression is a favourable prognostic factor (2023)
  • Author(s): Tanaka Ken、Miyoshi Hiroaki、Kawamoto Keisuke、Shimasaki Yasumasa、Nakashima Kazutaka、Imamoto Teppei、Yamada Kyohei、Takeuchi Mai、Moritsubo Mayuko、Furuta Takuya、Kohno Kei、Tamura Shinobu、Sonoki Takashi、Ohshima Koichi
  • Journal Title: Pathology Volume: 56 Pages: 81-91
  • DOI: 10.1016/j.pathol.2023.10.007
  • Peer Reviewed / Int'l Joint Research
• [Journal Article] Clinical Characteristics and Diagnostic Prediction of Severe Fever with Thrombocytopenia Syndrome and Rickettsiosis in the Co-Endemic Wakayama Prefecture, Japan. (2023)
  • Author(s): Teramoto K, Tamura S, Yoshida K, Inada Y, Yamashita Y, Morimoto M, Mushino T, Koreeda D, Miyamoto K, Komiya N, Nakano Y, Takagaki Y, Koizumi Y.
  • Journal Title: Medicina (Kaunas) Volume: 59(11) Issue: 11 Pages: 2024-2024
  • DOI: 10.3390/medicina59112024
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Angioimmunoblastic T-Cell Lymphoma after Treatment of Classic Hodgkin Lymphoma: A Case Report. (2023)
  • Author(s): Tanaka K, Miyoshi H, Yamashita Y, Iwamoto R, Yokoya Y, Tochino Y, Arakawa F, Tamura S, Murata SI, Sonoki T, Ohshima K.
  • Journal Title: Hematology Reports Volume: 15(4) Issue: 4 Pages: 662-669
  • DOI: 10.3390/hematolrep15040067
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Administration of combined venetoclax and azacitidine in a patient with acute myeloid leukemia and multiple comorbidities undergoing dialysis: A case report (2023)
  • Author(s): Akagi Yuina、Yamashita Yusuke、Kosako Hideki、Furuya Yoshiaki、Hosoi Hiroki、Mushino Toshiki、Murata Shogo、Nishikawa Akinori、Tamura Shinobu、Nakao Taisei、Sonoki Takashi
  • Journal Title: eJHaem Volume: 4 Issue: 3 Pages: 841-843
  • DOI: 10.1002/jha2.732
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Coagulation potentials of plasma‐derived factors VIIa and X mixture (Byclot^?) evaluated by global coagulation assay in patients with acquired haemophilia A (2023)
  • Author(s): Takeyama Masahiro、Furukawa Shoko、Ogiwara Kenichi、Tamura Shinobu、Ohno Hitoshi、Higasa Satoshi、Shimonishi Naruto、Nakajima Yuto、Onishi Tomoko、Nogami Keiji
  • Journal Title: Haemophilia Volume: 30 Issue: 1 Pages: 249-252
  • DOI: 10.1111/hae.14902
  • Peer Reviewed / Int'l Joint Research
• [Journal Article] An evaluation based on relative treatment intensity in older patients treated with reduced-dose R-THP-COP therapy for diffuse large B-cell lymphoma: A multicenter retrospective cohort study (2023)
  • Author(s): Hiroi Takayuki、Hosoi Hiroki、Kuriyama Kodai、Murata Shogo、Morimoto Masaya、Mushino Toshiki、Nishikawa Akinori、Tamura Shinobu、Sonoki Takashi
  • Journal Title: Journal of Geriatric Oncology Volume: 14 Issue: 1 Pages: 101396-101396
  • DOI: 10.1016/j.jgo.2022.10.011
  • Peer Reviewed / Open Access
• [Journal Article] Effect of SARS-CoV-2 BNT162b2 mRNA vaccine on thyroid autoimmunity: A twelve-month follow-up study (2023)
  • Author(s): Morita Shuhei、Takagi Tomoyuki、Inaba Hidefumi、Furukawa Yasushi、Kishimoto Shohei、Uraki Shinsuke、Shimo Naoki、Takeshima Ken、Uraki Saya、Doi Kei、Imagawa Mitsuyo、Kokawa Mika、Konami Tomomi、Hara Hitomi、Hara Yoshihiro、Sone Emiko、Furuta Hiroto、Nishi Masahiro、Doi Asako、Tamura Shinobu、Mastuoka Taka-aki
  • Journal Title: Frontiers in Endocrinology Volume: 14 Pages: 1058007-1058007
  • DOI: 10.3389/fendo.2023.1058007
  • Peer Reviewed / Open Access
• [Journal Article] B cell receptor repertoire analysis from autopsy samples of COVID-19 patients (2023)
  • Author(s): Iwabuchi Sadahiro、Shichino Shigeyuki、Imafuku Tadashi、Yamaji Kenzaburo、Miyamoto Kyohei、Tamura Shinobu、Ueha Satoshi、Ito Toshihiro、Murata Shin-ichi、Kondo Toshikazu、Ikeo Kazuho、Suzuki Yutaka、Matsushima Kouji、Kohara Michinori、Torigoe Toshihiko、Yamaue Hiroki、Hashimoto Shinichi
  • Journal Title: Frontiers in Immunology Volume: 14 Pages: 1034978-1034978
  • DOI: 10.3389/fimmu.2023.1034978
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Acquired Thrombotic Thrombocytopenic Purpura Following BNT162b2 mRNA Coronavirus Disease Vaccination in a Japanese Patient (2022)
  • Author(s): Yoshida Kikuaki、Sakaki Ayaka、Matsuyama Yoriko、Mushino Toshiki、Matsumoto Masanori、Sonoki Takashi、Tamura Shinobu
  • Journal Title: Internal Medicine Volume: 61 Issue: 3 Pages: 407-412
  • DOI: 10.2169/internalmedicine.8568-21
  • NAID: 130008150337
  • ISSN: 0918-2918, 1349-7235
  • Year and Date: 2022-02-01
  • Peer Reviewed / Open Access
• [Journal Article] Allosteric Inhibition of c-Abl to Induce Unfolded Protein Response and Cell Death in Multiple Myeloma (2022)
  • Author(s): Kosako Hideki、Yamashita Yusuke、Morita Shuhei、Iwabuchi Sadahiro、Hashimoto Shinichi、Matsuoka Taka-Aki、Sonoki Takashi、Tamura Shinobu
  • Journal Title: International Journal of Molecular Sciences Volume: 23 Issue: 24 Pages: 16162-16162
  • DOI: 10.3390/ijms232416162
  • Peer Reviewed / Open Access
• [Journal Article] Use of thromboelastography before the administration of hemostatic agents to safely taper recombinant activated factor VII in acquired hemophilia A: a report of three cases (2022)
  • Author(s): Hosoi Hiroki、Akagi Yuina、Mushino Toshiki、Takeyama Masahiro、Minoura Naoto、Hiroi Takayuki、Furuya Yoshiaki、Morimoto Masaya、Murata Shogo、Tamura Shinobu、Sonoki Takashi
  • Journal Title: Thrombosis Journal Volume: 20 Issue: 1 Pages: 28-28
  • DOI: 10.1186/s12959-022-00387-x
  • Peer Reviewed / Open Access
• [Journal Article] Sudden Cardiac Death in a Patient with Thrombotic Thrombocytopenic Purpura: A Case Report (2022)
  • Author(s): Yoshida Kikuaki、Murata Shogo、Morimoto Masaya、Mushino Toshiki、Tanaka Ken、Yamashita Yusuke、Hosoi Hiroki、Nishikawa Akinori、Tamura Shinobu、Hatakeyama Kinta、Matsumoto Masanori、Sonoki Takashi
  • Journal Title: Hematology Reports Volume: 14 Issue: 2 Pages: 203-209
  • DOI: 10.3390/hematolrep14020027
  • Peer Reviewed / Open Access
• [Journal Article] Renal Leukemic Infiltration Overlapping Acute Focal Bacterial Nephritis during Myelodysplastic Syndrome: An Autopsy Case Report (2022)
  • Author(s): Murakami Keishu、Tamura Shinobu、Maruyama Anna、Naitoh Tomomi、Teramoto Kan、Mikasa Yurina、Tanaka Masaoh、Murata Shinichi、Kato Seiya
  • Journal Title: Medicina Volume: 58 Issue: 8 Pages: 1060-1060
  • DOI: 10.3390/medicina58081060
  • Peer Reviewed / Open Access
• [Journal Article] Intestinal Mucosa-Associated Lymphoid Tissue Lymphoma Transforming into Diffuse Large B-Cell Lymphoma in a Young Adult Patient with Neurofibromatosis Type 1: A Case Report (2022)
  • Author(s): Kosako Hideki、Yamashita Yusuke、Tanaka Ken、Mishima Hiroyuki、Iwamoto Ryuta、Kinoshita Akira、Murata Shin-ichi、Ohshima Koichi、Yoshiura Koh-ichiro、Sonoki Takashi、Tamura Shinobu
  • Journal Title: Medicina Volume: 58 Issue: 12 Pages: 1830-1830
  • DOI: 10.3390/medicina58121830
  • Peer Reviewed / Open Access
• [Journal Article] Risk Factors for Therapeutic Intervention of Remdesivir in Mild to Moderate COVID-19?A Single-Center Retrospective Study of the COVID-19 Fourth Pandemic Period in Wakayama, Japan (2022)
  • Author(s): Tamura Shinobu、Kaki Takahiro、Niwa Mayako、Yamano Yukiko、Kawai Shintaro、Yamashita Yusuke、Tanaka Harumi、Saito Yoshinobu、Kajimoto Yoshinori、Koizumi Yusuke、Yamaue Hiroki、Nakao Naoyuki、Nojiri Takako、Hironishi Masaya
  • Journal Title: Medicina Volume: 58 Issue: 1 Pages: 118-118
  • DOI: 10.3390/medicina58010118
  • Peer Reviewed / Open Access
• [Journal Article] Multisystem inflammatory syndrome in adults after acute coronavirus disease 2019 in a Japanese woman: A case report (2022)
  • Author(s): Miyamoto Kyohei、Koizumi Yusuke、Tamura Shinobu、Nakashima Tsuyoshi、Kobai Kaori、Tanaka Rikako、Shibata Mami、Shima Nozomu、Nemoto Shigeki、Fukumoto Nobu、Kato Seiya
  • Journal Title: Journal of Infection and Chemotherapy Volume: 28 Issue: 1 Pages: 99-102
  • DOI: 10.1016/j.jiac.2021.09.014
  • Peer Reviewed / Open Access
• [Journal Article] Composite Epstein-Barr Virus-associated T-lymphoblastic and Peripheral T-cell Lymphomas: A Clonal Study (2021)
  • Author(s): Hosoi Hiroki、Mushino Toshiki、Nakashima Kazutaka、Kuriyama Kodai、Tamura Shinobu、Murata Shogo、Imadome Ken-Ichi、Ohshima Koichi、Sonoki Takashi
  • Journal Title: Internal Medicine Volume: 60 Issue: 13 Pages: 2119-2123
  • DOI: 10.2169/internalmedicine.6572-20
  • NAID: 130008060437
  • ISSN: 0918-2918, 1349-7235
  • Year and Date: 2021-07-01
  • Peer Reviewed / Open Access
• [Journal Article] Progressive Massive Splenomegaly in an Adult Patient with Kabuki Syndrome Complicated with Immune Thrombocytopenic Purpura (2021)
  • Author(s): Mushino Toshiki、Hiroi Takayuki、Yamashita Yusuke、Suzaki Norihiko、Mishima Hiroyuki、Ueno Masaki、Kinoshita Akira、Minami Koichi、Imai Kohsuke、Yoshiura Ko-ichiro、Sonoki Takashi、Tamura Shinobu
  • Journal Title: Internal Medicine Volume: 60 Issue: 12 Pages: 1927-1933
  • DOI: 10.2169/internalmedicine.6694-20
  • NAID: 130008052635
  • ISSN: 0918-2918, 1349-7235
  • Year and Date: 2021-06-15
  • Peer Reviewed / Open Access
• [Journal Article] Disseminated carcinomatosis of the bone marrow caused by prostate cancer diagnosed with only bone marrow biopsy (2021)
  • Author(s): Wakamiya Takahito、Tamura Shinobu、Kojima Fumiyoshi、Kohjimoto Yasuo、Hara Isao
  • Journal Title: IJU Case Reports Volume: 4 Issue: 5 Pages: 303-306
  • DOI: 10.1002/iju5.12332
  • Peer Reviewed / Open Access
• [Journal Article] Remote vital signs data monitoring during home blood transfusion: A pilot study (2021)
  • Author(s): Nishikawa Akinori、Fujimori Yoshihiro、Sakano Noriko、Mushino Toshiki、Tamura Shinobu、Kasahara Shingo、Akasaka Hiroshi、Sonoki Takashi
  • Journal Title: Health Science Reports Volume: 4 Issue: 3
  • DOI: 10.1002/hsr2.380
  • Peer Reviewed / Open Access
• [Journal Article] Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab (2021)
  • Author(s): Akagi Yuina、Kato Takashi、Yamashita Yusuke、Hosoi Hiroki、Murata Shogo、Yamamoto Shuto、Warigaya Kenji、Nakao Taisei、Murata Shinichi、Sonoki Takashi、Tamura Shinobu
  • Journal Title: Medicina Volume: 57 Issue: 9 Pages: 971-971
  • DOI: 10.3390/medicina57090971
  • Peer Reviewed / Open Access
• [Journal Article] Heterozygous missense variant of the proteasome subunit β-type 9 causes neonatal-onset autoinflammation and immunodeficiency (2021)
  • Author(s): Kanazawa N, Hemmi H, Kinjo N, Ohnishi H, Hamazaki J, M.H, K.A, M.T, H.S, K.N, K.S, H.Y, I.K, N.R, T.M, Y.Y, T.S, O.T, O.T, K.T, S.I, F.Y, W.N, I.Y, K.K, O.S, T.T, N.K, M.S, Y.K, Kaisho T
  • Journal Title: Nature Communications Volume: 12 Issue: 1 Pages: 0-0
  • DOI: 10.1038/s41467-021-27085-y
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Nonbacterial Thrombotic Endocarditis Associated with Acute Promyelocytic Leukemia: An Autopsy Case Report (2021)
  • Author(s): Hashimoto Tadayuki、Aoki Tatsuya、Kawabata Yoshitaka、Owai Yoshihiro、Matsuda Yoshikazu、Tamura Shinobu
  • Journal Title: Medicina Volume: 57 Issue: 11 Pages: 1264-1264
  • DOI: 10.3390/medicina57111264
  • Peer Reviewed / Open Access
• [Journal Article] A cluster of BK polyomavirus‐associated hemorrhagic cystitis after allogeneic hematopoietic stem cell transplantation (2021)
  • Author(s): Hosoi Hiroki、Murata Shogo、Suzuki Tetsuro、Li Tian‐Cheng、Hatanaka Kazuo、Tanaka‐Taya Keiko、Mushino Toshiki、Kuriyama Kodai、Tamura Shinobu、Hanaoka Nobuyoshi、Sonoki Takashi
  • Journal Title: Transplant Infectious Disease Volume: 23 Issue: 6
  • DOI: 10.1111/tid.13736
  • Peer Reviewed / Open Access
• [Journal Article] A Patient with Kabuki Syndrome Mutation Presenting with Very Severe Aplastic Anemia (2021)
  • Author(s): Tamura Shinobu、Kosako Hideki、Furuya Yoshiaki、Yamashita Yusuke、Mushino Toshiki、Mishima Hiroyuki、Kinoshita Akira、Nishikawa Akinori、Yoshiura Ko-Ichiro、Sonoki Takashi
  • Journal Title: Acta Haematologica Volume: 145 Issue: 1 Pages: 89-96
  • DOI: 10.1159/000518227
  • Peer Reviewed
• [Journal Article] Pegcetacoplan for refractory paroxysmal nocturnal haemoglobinuria associated with the C5 genetic variant (2021)
  • Author(s): Tamura Shinobu、Hori Yoshikazu、Hiroi Takayuki、Morimoto Masaya、Yamashita Yusuke、Mushino Toshiki、Sonoki Takashi
  • Journal Title: British Journal of Haematology Volume: 196 Issue: 6
  • DOI: 10.1111/bjh.17963
  • Peer Reviewed / Open Access
• [Journal Article] Targeting Adaptive IRE1α Signaling and PLK2 in Multiple Myeloma: Possible Anti-Tumor Mechanisms of KIRA8 and Nilotinib. (2020)
  • Author(s): Yamashita Y, Morita S, Hosoi H, Kobata H, Kishimoto S, Ishibashi T, Mishima H, Kinoshita A, Backes BJ, Yoshiura KI, Papa FR, Sonoki T, Tamura S
  • Journal Title: Int J Mol Sci. Volume: Aug 31;21(17) Issue: 17 Pages: 6314-6314
  • DOI: 10.3390/ijms21176314
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Consolidation with 90 Yttrium-ibritumomab tiuxetan after bendamustine and rituximab for relapsed follicular lymphoma (2020)
  • Author(s): Katsuhiro Miura , Hideki Tsujimura , Yasufumi Masaki , Tadashi Yoshino et al
  • Journal Title: Hematol Oncol Volume: Sep 25 Issue: 1 Pages: 1-10
  • DOI: 10.1002/hon.2809
  • Peer Reviewed / Int'l Joint Research
• [Journal Article] Safety and efficacy of anti-programmed cell death-1 monoclonal antibodies before and after allogeneic hematopoietic cell transplantation for relapsed or refractory Hodgkin lymphoma: a multicenter retrospective study (2020)
  • Author(s): Ito Ayumu、（15名）、Takemura Tomonari、Terakura Seitaro、Iida Hiroatsu、Nakamae Mika、Higuchi Kohei、Tamura Shinobu、Yoshioka Satoshi、Togitani Kazuto、Kawano Noriaki、Suzuki Ritsuro、Suzumiya Junji、Izutsu Koji、Teshima Takanori、Fukuda Takahiro
  • Journal Title: International Journal of Hematology Volume: 112 Issue: 5 Pages: 674-689
  • DOI: 10.1007/s12185-020-02960-4
  • Peer Reviewed / Int'l Joint Research
• [Journal Article] Two Episodes of Transfusion-related Acute Lung Injury (TRALI) Occurring within a Short Period (2020)
  • Author(s): Akagi Yuina、Murata Shogo、Yamashita Yusuke、Tanaka Ken、Hiroi Takayuki、Mushino Toshiki、Hosoi Hiroki、Nishikawa Akinori、Tamura Shinobu、Sonoki Takashi
  • Journal Title: Internal Medicine Volume: 59 Issue: 20 Pages: 2577-2581
  • DOI: 10.2169/internalmedicine.4700-20
  • NAID: 130007926297
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Brentuximab vedotin for refractory anaplastic lymphoma kinase-negative anaplastic large cell lymphoma in leukemic phase with <em>RUNX3</em> overexpression (2020)
  • Author(s): Yamashita Yusuke、Hori Yoshikazu、Kosako Hideki、Oiwa Takehiro、Warigaya Kenji、Mushino Toshiki、Murata Shogo、Fujimoto Masakazu、Nishikawa Akinori、Murata Shin-ichi、Sonoki Takashi、Tamura Shinobu
  • Journal Title: Hematology Reports Volume: 12 Issue: 1 Pages: 8368-8368
  • DOI: 10.4081/hr.2020.8368
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Paroxysmal nocturnal hemoglobinuria treated with eculizumab in Wakayama, Japan: a retrospective analysis (2020)
  • Author(s): 田村 志宣、花岡 伸佳、園木 孝志、古家 美昭、堀 善和、弘井 孝幸、山下 友佑、大岩 健洋、村田 祥吾、蒸野 寿紀、西川 彰則
  • Journal Title: Rinsho Ketsueki Volume: 61 Issue: 6 Pages: 605-611
  • DOI: 10.11406/rinketsu.61.605
  • NAID: 130007869147
  • ISSN: 0485-1439, 1882-0824
  • Peer Reviewed
• [Presentation] Mutant mice carrying a novel hypomorphic mutation of the DNA ligase IV gene show Th1-skewing intestinal inflammations with severe adaptive immunodeficiency (2023)
  • Author(s): Yamashita Y, Kato T, Kosako H, Iwabuchi S, Sasaki I, Kanazawa N, Hemmi H, Hashimoto S, Sonoki T, Tamura S, Kaisho T
  • Organizer: 12th International Society of Systemic Autoinflammatory Diseases Meeting
  • Int'l Joint Research
• [Presentation] LIG4 syndrome mutation can lead to Th1-skewing intestinal inflammation in mice (2023)
  • Author(s): Tamura S, Yamashita Y, Kato T, Kosako H, Iwabuchi S, Sasaki I, Hemmi H, Hashimoto S, Kaisho T
  • Organizer: 2nd International Symposium of Clinical Immunology Meeting
  • Int'l Joint Research
• [Presentation] Hypomorphic mutation in a DNA damage repair system causes Th1-skewing intestinal inflammation with severe combined immunodeficiency (2023)
  • Author(s): Tamura S, Yamashita Y, Kato T, Kosako H, Iwabuchi S, Sasaki I, Hemmi H, Hashimoto S, Kaisho T
  • Organizer: 29th International Symposium on Molecular Cell Biology of Macrophages
  • Int'l Joint Research
• [Presentation] Impaired DNA double-strand break repair can drive innate immunity response in mice (2023)
  • Author(s): Tamura S, Kosako H, Yamashita Y, Kato T, Tabata S, Iwabuchi S, Tanaka K, Nakashima K, Sasaki I, Ohshima K, Hashimoto S, Sonoki T, Kaisho T
  • Organizer: 13th JSH International Symposium
  • Int'l Joint Research
• [Presentation] A novel Lig4 hypomorphic mutation in mice can lead to intestinal inflammation evoked by skewing Th1 (2023)
  • Author(s): Yamashita Y, Kosako H, Kato T, Tabata S, Iwabuchi S, Tanaka K, Nakashima K, Sasaki I, Ohshima K, Hashimoto S, Sonoki T, Kaisho T, Tamura S
  • Organizer: 13th JSH International Symposium
• [Presentation] Asciminib Induces Anti-Myeloma Effects Through Terminal Unfolded Protein Response (2023)
  • Author(s): Kosako H, Yamashita Y, Morita S, Iwabuchi S, Tamura S, Hashimoto S, Sonoki T
  • Organizer: 13th JSH International Symposium
  • Int'l Joint Research
• [Presentation] Lig4 遺伝子の低形成性変異を有したマウスは異常Th1細胞による炎症性腸疾患を発症する (2023)
  • Author(s): 田村志宣, 山下友佑, 小浴秀樹, 加藤喬, 岩淵禎弘, 佐々木泉, 橋本真一, 改正恒康
  • Organizer: 第6 回日本免疫不全・自己炎症学会総会・学術集会
• [Presentation] Hypomorphic mutation of Lig4 gene in mice predisposes to intestinal inflammation driven by Th1 cells (2022)
  • Author(s): 山下友佑, 加藤喬, 小浴秀樹, 岩淵禎弘, 田中顕, 中嶋一貴, 佐々木泉, 橋本真一, 大島孝一, 改正恒康, 園木孝志, 田村志宣
  • Organizer: 第84回日本血液学会学術集会
• [Presentation] Th1-skewing colitis in mutant mice with homozygous missense mutation of DNA Ligase IV. (2021)
  • Author(s): Tamura S, Yamashita Y, Orimo T, Iwabuchi S, Nakashima K, Sasaki I, Hemmi H, Hashimoto S, Ohshima K, Kaisho T, Sonoki T
  • Organizer: The 27th International Symposium on Molecular Cell Biology of Macrophages
  • Int'l Joint Research
• [Presentation] Blockade of IRE1α signaling exhibits antimyeloma effects via downregulation of Polo-like kinase 2. (2021)
  • Author(s): Tamura S, Yamashita Y, Morita S, Sonoki T
  • Organizer: 第83回日本血液学会学術集会
• [Presentation] Pathological characterization of Th1-mediated colitis based on primary immunodeficiency. (2020)
  • Author(s): Tamura S, Yamashita Y, Orimo T, Iwabuchi S, Nakashima K, Sasaki I, Hemmi H, Hashimoto S, Ohshima K, Kaisho T, Sonoki T
  • Organizer: 第82回日本血液学会学術集会