Underlying mechanisms for immune disorders caused by proteasome dysfunctions

• Project/Area Number: 21K19384
• Research Category: Grant-in-Aid for Challenging Research (Exploratory)
• Allocation Type: Multi-year Fund
• Review Section: Medium-sized Section 49:Pathology, infection/immunology, and related fields
• Research Institution: Wakayama Medical University
• Principal Investigator: Kaisho Tsuneyasu 和歌山県立医科大学, 先端医学研究所, 教授 (60224325)
• Co-Investigator(Kenkyū-buntansha): 金澤 伸雄 兵庫医科大学, 医学部, 教授 (90343227)
• Project Period (FY): 2021-07-09 – 2023-03-31
• Project Status: Completed (Fiscal Year 2022)
• Budget Amount: ¥6,500,000 (Direct Cost: ¥5,000,000、Indirect Cost: ¥1,500,000); Fiscal Year 2022: ¥2,600,000 (Direct Cost: ¥2,000,000、Indirect Cost: ¥600,000); Fiscal Year 2021: ¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
• Keywords: プロテアソーム / タンパク質分解 / 自己炎症 / 免疫不全 / 自己炎症性疾患 / 遺伝子改変マウス

Outline of Research at the Start

プロテアソームは、不要あるいは不良のタンパク質の分解除去を担うタンパク質複合体であり、そのサブユニットの遺伝子バリアントにより、自己炎症性疾患をきたす。しかし、適切なモデルマウスがないため、その病態についてはほとんどわかっていない。本研究では、プロテアソームサブユニットの新規の遺伝子バリアントを持つマウスの解析を進め、そのマウスで認められたユニークな免疫病態の分子基盤を解明する。

Outline of Final Research Achievements

A novel heterozygous missense variant (p.G156D variant) was commonly detected in the gene encoding the proteasome subunit beta1i in two patients showing autoinflammatory manifestations with acquired immune deficiency. This variant was introduced into mice and analyzed. In the heterozygous mutant mice, acquired immunodeficiency was reproduced and, concerning the innate immune cells, monocytes and neutrophils were increased in number. The results led us to propose a new disease category, proteasome-associated autoinflammatory syndrome with immunodeficiency.

Academic Significance and Societal Importance of the Research Achievements

プロテアソームサブユニットの新規の遺伝子バリアントが同定され、そのバリアントを持つマウスにおいてヒト病態が再現された。プロテアソームの機能異常は、稀少疾患ばかりでなく、がん、神経変性、老化などcommon diseaseにおいてもみとめられる。本研究で樹立された遺伝子改変マウスの更なる解析により、プロテアソーム機能異常により生じる様々な病態解明が進むことが期待される。

Research Products

• [Int'l Joint Research] Yale Univ./Harverd Medical School/Emory Univ.(米国)
• [Int'l Joint Research] Univ. of Melbourne/Australia National Univ./Telethon Kids Institute(オーストラリア)
• [Int'l Joint Research] Mainz Univ./Univ. Hospital Wuerzburg/Univ. Hostital Cologne(ドイツ)
• [Int'l Joint Research] London Research Institute/Imperial College/Cardiff Univ.(英国)
• [Int'l Joint Research] San Raffaele Scientific Institute/ICGEB(イタリア)
• [Int'l Joint Research]
• [Int'l Joint Research] University of New South Wales/The University of Western Australia(オーストラリア)
• [Int'l Joint Research] Indiana University/H. Lee Moffitt Cancer Center(米国)
• [Int'l Joint Research] Ludwig Maximilians Universitat Munchen(ドイツ)
• [Journal Article] XCR1+ DCs are critical for T cell-mediated immunotherapy of chronic viral infections. (2023)
  • Author(s): E. Domenjo Vila, V. Casella, R. Iwabuchi, E. Fossum, M. Pedragosa, Q. Castellvi, P. Cebollada Rica, T. Kaisho, K. Terahara, G. Bocharov, J. Argilaguet, A. Meyerhans.
  • Journal Title: Cell Rep Volume: 42 Issue: 2 Pages: 112123-112123
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• [Journal Article] 免疫不全を伴うプロテアソーム関連自己炎症症候群. Proteasome-associated autoinflammatory syndrome with immunodeficiency. (2023)
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• [Journal Article] Conventional Type 1 Dendritic Cells in Intestinal Immune Homeostasis. (2022)
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• [Journal Article] Preparation and Inflammasome Activation of Murine Bone Marrow-Derived and Resident Peritoneal Macrophages (2022)
  • Author(s): Sasaki I, Kaisho T.
  • Journal Title: Methods Mol Biol. Volume: 2427 Pages: 95-104
  • DOI: 10.1007/978-1-0716-1971-1_9
  • ISBN: 9781071619704, 9781071619711
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• [Journal Article] Type 1 conventional dendritic cells maintain and guide the differentiation of precursors of exhausted T cells in distinct cellular niches (2022)
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  • Journal Title: Immunity Volume: 55 Issue: 4 Pages: 656-670
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  • Journal Title: JCI Insight Volume: 7 Issue: 7 Pages: 151593-151593
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• [Journal Article] The liver contains distinct interconnected networks of CX3CR1+ macrophages, XCR1+ type 1 and CD301a+ type 2 conventional dendritic cells embedded within portal tracts (2022)
  • Author(s): K. English, S. Yang Tan, R. Kwan, L. E Holz, F. Sierro, C. McGuffog, T. Kaisho, W. R Heath, K. Pa MacDonald, G. W McCaughan, D. G Bowen, P. Bertolino.
  • Journal Title: Immunol Cell Biol Volume: 100 Issue: 6 Pages: 394-408
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  • Journal Title: Nature Volume: 606 Issue: 7916 Pages: 992-998
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• [Journal Article] Thymic epithelial cells co-opt lineage-defining transcription factors to eliminate autoreactive T cells (2022)
  • Author(s): D. A Michelson, K. Hase, T. Kaisho, C. Benoist, D. Mathis.
  • Journal Title: Cell Volume: 185 Issue: 14 Pages: 2542-2558
  • DOI: 10.1016/j.cell.2022.05.018
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• [Journal Article] Depletion of cDC1 cells upon immunotherapy of established tumors precludes efficacy. Cancer Research Depletion of Conventional Type-1 Dendritic Cells in Established Tumors Suppresses Immunotherapy Efficacy (2022)
  • Author(s): A. Teijeira, S. Garasa, C. luri, C. de Andrea, M. Gato, C. Molina, T. Kaisho, A. Cirella, A. Azpilikueta, S. Wculek, J. Egea, I. Olivera, I. Rodriguez, A. Rouzaut, V. Verkhusha, K. Valencia, D. Sancho, P. Berraondo, I. Melero.
  • Journal Title: Cancer Research Volume: 82 Issue: 23 Pages: 4373-4385
  • DOI: 10.1158/0008-5472.can-22-1046
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• [Journal Article] Spi-B alleviates food allergy by securing mucosal barrier and immune tolerance in the intestine. (2022)
  • Author(s): N. Ishihara,Y. Nakamura, K. Yakabe, S. Komiyama, Y. Fujimura, T. Kaisho, S. Kimura, K. Hase.
  • Journal Title: Front Allergy Volume: 3 Pages: 996657-996657
  • DOI: 10.3389/falgy.2022.996657
  • Peer Reviewed / Open Access
• [Journal Article] Tmem45b is essential for inflammation- and tissue injury?induced mechanical pain hypersensitivity (2022)
  • Author(s): Tanioku Tadashi、Nishibata Masayuki、Tokinaga Yasuyuki、Konno Kohtaro、Watanabe Masahiko、Hemmi Hiroaki、Fukuda-Ohta Yuri、Kaisho Tsuneyasu、Furue Hidemasa、Kawamata Tomoyuki
  • Journal Title: Proceedings of the National Academy of Sciences Volume: 119 Issue: 45 Pages: 119-119
  • DOI: 10.1073/pnas.2121989119
  • Peer Reviewed / Open Access
• [Journal Article] Androgen-regulated MafB drives cell migration via MMP11-dependent extracellular matrix remodeling in mice (2022)
  • Author(s): Alcantara Mellissa C.、Suzuki Kentaro、Acebedo Alvin R.、Kajioka Daiki、Hirohata Satoshi、Kaisho Tsuneyasu、Hatano Yu、Yamagata Kazuo、Takahashi Satoru、Yamada Gen
  • Journal Title: iScience Volume: 25 Issue: 12 Pages: 105609-105609
  • DOI: 10.1016/j.isci.2022.105609
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Immunogenic tumor cell death promotes dendritic cell migration and inhibits tumor growth via enhanced T cell immunity (2021)
  • Author(s): Moriya Taiki、Kitagawa Kurumi、Hayakawa Yuuki、Hemmi Hiroaki、Kaisho Tsuneyasu、Ueha Satoshi、Ikebuchi Ryoyo、Yasuda Ippei、Nakanishi Yasutaka、Honda Tetsuya、Matsushima Koji、Kabashima Kenji、Ueda Mizuki、Kusumoto Yutaka、Chtanova Tatyana、Tomura Michio
  • Journal Title: iScience Volume: 24 Issue: 5 Pages: 102424-102424
  • DOI: 10.1016/j.isci.2021.102424
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] Augmentation of STING‐induced type I interferon production in COPA syndrome (2021)
  • Author(s): Kato Takashi、Yamamoto Masaki、Honda Yoshitaka、Orimo Takashi、Sasaki Izumi、Murakami Kohei、Hemmi Hiroaki、Fukuda‐Ohta Yuri、Isono Kyoichi、Takayama Saki、Nakamura Hidenori、Otsuki Yoshiro、Miyamoto Toshiaki、Takita Junko、Yasumi Takahiro、Nishikomori Ryuta、Matsubayashi Tadashi、Izawa Kazushi、Kaisho Tsuneyasu
  • Journal Title: Arthritis & Rheumatology Volume: in press Issue: 11 Pages: 2105-2115
  • DOI: 10.1002/art.41790
  • Peer Reviewed / Open Access
• [Journal Article] The inhibitory receptor TIM-3 limits activation of the cGAS-STING pathway in intra-tumoral dendritic cells by suppressing extracellular DNA uptake (2021)
  • Author(s): de Mingo Pulido Alvaro, et al. 全著者16名中10番目
  • Journal Title: Immunity Volume: S1074-7613 Issue: 6 Pages: 00181-3
  • DOI: 10.1016/j.immuni.2021.04.019
  • Peer Reviewed / Int'l Joint Research
• [Journal Article] Type I interferon mediated induction of somatostatin leads to suppression of ghrelin and appetite thereby promoting viral immunity in mice (2021)
  • Author(s): Stutte Susanne、Ruf Janina、Kugler Ina、Ishikawa-Ankerhold Hellen、Parzefall Andreas、Marconi Peggy、Maeda Takahiro、Kaisho Tsuneyasu、Krug Anne、Popper Bastian、Lauterbach Henning、Colonna Marco、von Andrian Ulrich、Brocker Thomas
  • Journal Title: Brain, Behavior, and Immunity Volume: S0889-1591 Pages: 00176-8
  • DOI: 10.1016/j.bbi.2021.04.018
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Journal Article] IFNβ Is a Potent Adjuvant for Cancer Vaccination Strategies. (2021)
  • Author(s): K. M. Audsley, T. Wagner, C. Ta, H. V. Newnes, A. C. Buzzai, S. A. Barnes, B. Wylie, J. Armitage, T. Kaisho, A. Bosco, A. McDonnell, M. Cruickshank, V. S. Fear, B. Foley, J. Waithman
  • Journal Title: Frontiers Immunol Volume: 12 Pages: 735133-735133
  • DOI: 10.3389/fimmu.2021.735133
  • Peer Reviewed / Open Access / Int'l Joint Research
• [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] プロテアソーム機能不全による自己炎症. (2021)
  • Author(s): 改正恒康
  • Journal Title: 炎症と免疫 Volume: 29 Pages: 195-200
• [Presentation] Hypomorphic mutation of Lig4 gene in mice predisposes to intestinal inflammation driven by Th1 cells. (2022)
  • Author(s): Y. Yamashita, T. Kato, H. Kosako, S. Iwabuchi, K. Tanaka, K. Nakashima, I. Sasaki, S. Hashimoto, K. Ohshima, T. Kaisho, T. Sonoki, S. Tamura.
  • Organizer: 第84回日本血液学会学術集会
• [Presentation] Model mice of human inborn errors of immunity showing mixed manifestations of aouoinflammation,immunodeficiency and/or autoimmunity. (2022)
  • Author(s): T. Kaisho.
  • Organizer: 第51回日本免疫学会総会・学術集会
  • Invited
• [Presentation] γδT cells in Peyer’s patches acquire the encephalitogenic phenotype through the activation by commensals and exacerbates autoimmune ncephaomyelitis. (2022)
  • Author(s): S. Komiyama, D. Takahashi, T.Kaisho, K. Hase.
  • Organizer: 第51回日本免疫学会総会・学術集会
• [Presentation] Dendritic and T cell abnormalities in an autoinflammatory disease with interstitial lung disease and type 1 interferonopathy caused by a disability of retrongrade protein trafficking. (2022)
  • Author(s): T. Kato, I.Sasaki, H. Hemmi, Y. Honda. K. Izawa, R. Nishikomori, T. Kaisho.
  • Organizer: 第51回日本免疫学会総会・学術集会
• [Presentation] Type 1 conventional dendritic cells of bone marrow assistthe prograession of multiple myeloma and consist of tumor microenvironment in the transplanted Vk*MYC mouse model. (2022)
  • Author(s): S.Suzuki, S.Tsuda, T.Takubo, H.Toyoshima, M.Yoshino, T.Kaisho, T.Fukuda, K.Tokoyoda.
  • Organizer: 第51回日本免疫学会総会・学術集会
• [Presentation] The role of Rnase T2 in macrophage homeostasis. (2022)
  • Author(s): R. Sato, K.Liu, T.Shibata, R.Fukui, K.Hoshino, T.Kaisho, K.Miyake.
  • Organizer: 第51回日本免疫学会総会・学術集会
• [Presentation] 遺伝子改変マウスを用いた組織常在マクロファージの免疫学的解析 (2022)
  • Author(s): 加治汐梨、佐々木泉、加藤喬、西山奈央子、山本明日美、脇村一輝、北野雅之、改正恒康
  • Organizer: 第29回日本免疫毒性学会学術年会
• [Presentation] Activation of type 1 interferon signaling in dendritic cells and T cells of the mouse model of COPA syndrome. (2022)
  • Author(s): T.Kato, R.Yamasaki, N.W-Nishiyama. I.Sasaki, S.Kaji, K.Murakami, H.Hemmi, Y.Honda, K.Izawa, Y.Otsuki, T.Matsubayashi, R.Nishikomori, T.Kaisho.
  • Organizer: 第29回日本免疫毒性学会学術年会
• [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 (2022)
  • Author(s): 田村志宜、山下友佑、小浴秀樹、加藤喬、岩淵貞弘、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] Immunotherapy of chronic virus infections: exhausted CD8+ T cell subsets are differentially regulated by XCR1+ DC. (2022)
  • Author(s): A.Meyerhans, E.D-Vila, V.Casella, R.Iwabuxhi, E.Fossum, M.Pedragosa, Q.Castellvi, T.Kaisho, K.Terahara, G.Bocharov, J.Argilaguet.
  • Organizer: The 16th International Symposium on Dendritic Cells 2022
  • Int'l Joint Research
• [Presentation] γδT17 cells in Peyer’s patches acquire the encephalitogenic phenotype through the activation by commensals. (2022)
  • Author(s): S. Komiyama, D. Takahashi, T. Kaisho, H. Watarai, K. Hase.
  • Organizer: The 9th Congress of International Human Microbiome Consortium
  • Int'l Joint Research
• [Presentation] がん免疫療法における抗原提示細胞の意義 (2021)
  • Author(s): 改正恒康
  • Organizer: 第25回日本がん免疫学会総会
  • Invited
• [Presentation] 自己炎症性疾患COPA症候群変異マウスにおけるSTINGシグナルの活性化 (2021)
  • Author(s): 加藤喬、折茂貴是、大田（福田）有里、西山奈央子、中井千尋、張江伊水、田中日向子、佐々木泉、村上康平、邊見弘明、本田吉孝、井澤和司、西小森隆太、改正恒康
  • Organizer: 第85回日本インターフェロン・サイトカイン学会学術集会
• [Presentation] XCR1陽性樹状細胞分化におけるプロテアソームの役割 (2021)
  • Author(s): 西山奈央子、佐々木泉、邊見弘明、改正恒康
  • Organizer: 第25回日本がん免疫学会総会
• [Presentation] XCR1+DCをターゲットにした whole-cell-vaccineの開発 (2021)
  • Author(s): 宮本篤、勝田将裕、宮澤基樹、北畑裕司、水本有紀、小林良平、尾島敏康、邊見弘明、戸村道夫、改正恒康、山上裕機
  • Organizer: 第25回日本がん免疫学会総会
• [Presentation] 樹状細胞サブセット(XCRl+DC)への特異的送達によるがんペプチドワクチン療法 (2021)
  • Author(s): 水本有紀、勝田将裕、宮澤基樹、北畑裕司、宮本篤、中森幹人、松田健司、尾島敏康、邊見弘明、玉田耕治、改正恒康、山上裕機
  • Organizer: 第25回日本がん免疫学会総会
• [Presentation] 遺伝子改変マウスを用いたCOPA症候群の病態解明 (2021)
  • Author(s): 加藤喬、川野果歩、折茂貴是、大田（福田）有里、西山奈央子、中井千尋、佐々木泉、改正恒康
  • Organizer: 第89回和歌山医学会総会
• [Presentation] 自己炎症性疾患COPA症候群モデルマウスにおけるI型IFN症発症の分子基盤 (2021)
  • Author(s): 加藤喬、折茂貴是、大田（福田）有里、西山奈央子、中井千尋、佐々木泉、村上康平、邊見弘明、本田吉孝、井澤和司、大月寛郎、松林正、西小森隆太、改正恒康
  • Organizer: 第28回日本免疫毒性学会学術集会
• [Presentation] Unique location in the immunoproteasome complex of a variant causing proteasome-associated autoinflammatory syndrome with immunodeficiency (2021)
  • Author(s): N. Kanazawa, H. Hemmi, N. Kinjo, H. Ohnishi, J. Hamazaki, H. Mishima, A. Kinoshita, T. Mizushima, S. Murata, K. Yoshiura, T. Kaisho
  • Organizer: 第50回日本免疫学会総会
• [Presentation] Hyperactivation of STING-induced type I interferon pathway in dendritic cells from novel mice model for an autoinflammatory disease, COPA syndrome (2021)
  • Author(s): T. Kato, T. Orimo, Y. Fukuda-Ohta, I. Sasaki, H. Hemmi, Y. Honda, K. Izawa, R. Nishikomori, T. Kaisho
  • Organizer: 第50回日本免疫学会総会
• [Presentation] Impaired development of myeloid cells in proteasome subunit mutant mice (2021)
  • Author(s): H. Hemmi, T. Orimo, I. Sasaki, T. Kato, Y. Fukuda-Ohta, N. Kinjo, H. Ohnishi, N. Kanazawa, T. Kaisho
  • Organizer: 第50回日本免疫学会総会
• [Presentation] Sublingual dendritic cell (DC) - T cell clusters and distribution of DCs in the oral cavity (2021)
  • Author(s): Y. Kusumoto, T. Kaisho, H. Hemmi, T. Katakai, T. Honda, J. Kikuta, K. Kataoka, T. Moriya, M. Ishii, K. Kabashima, M. Tomura
  • Organizer: 第50回日本免疫学会総会
• [Presentation] COPA症候群の新規モデルマウスにおけるSTINGの活性化とT細胞の異常. Activation of STING and T cell abnormalities in a novel mouse model of COPA syndrome (2021)
  • Author(s): 加藤喬、折茂貴是、大田(福田)有里、西山奈央子、中井千尋、佐々木泉、村上康平、邊見弘明、本田吉孝、井澤和司、八角高裕、大月寛郎、松林正、西小森隆太、改正恒康
  • Organizer: 第5回日本免疫不全・自己炎症学会総会・学術集会
• [Presentation] Chemokine-medicated delivery of cancer peptide vaccine to a dendritic cell subset with high crosspresentation activity (2021)
  • Author(s): Y. Mizumoto, H. Hemmi, M. Katsuda, Y. Fukuda-Ohta, T. Orimo, I. Sasaki, K. Tamada, H. Yamaue, T. Kaisho
  • Organizer: The 27th International Symposium on Molecular Cell Biology of Macrophage (MMCB 2020+1)
  • Int'l Joint Research
• [Presentation] Impaired monocyte and dendritic cell development in mice carrying a mutation identified from the proteasome-associated autoinflammation and immunodeficiency disease patient. (2021)
  • Author(s): H. Hemmi, T. Orimo, Naoko Nishiyama, T. Kato, T. Ozasa, I. Sasaki, H. Ohnishi, N. Kinjo, N. Kanzawa, T. Kaisho
  • Organizer: The 27th International Symposium on Molecular Cell Biology of Macrophage (MMCB 2020+1)
  • Int'l Joint Research
• [Presentation] Interstitial Lung Disease and abnormal STING activation in COPA V242G heterozygous variant mice (2021)
  • Author(s): T. Kato, T. Orimo, Y. Fukuda-Ohta, N. Wakaki-Nishiyama, C. Nakai, I. Sasaki, K. Murakami, H. Hemmi, Y. Honda, K. Izawa, Y. Otsuki, T. Matsubayashi, R. Nishikomori, T. Kaisho
  • Organizer: IEIIS2021: 16th Meeting of the International Endotoxin and Innate Immunity Society (26tth Japanese Endotoxin and innate Immunity Society)
  • Int'l Joint Research
• [Presentation] Analysis of innate immune cells in proteasome subunit mutant mice (2021)
  • Author(s): H. Hemmi, T. Orimo, I. Sasaki, T. Kato, Y. Fukuda-Ohta, N. Kinjo, H. Ohnishi, N. Kanzawa, T. Kaisho
  • Organizer: IEIIS2021: 16th Meeting of the International Endotoxin and Innate Immunity Society (26tth Japanese Endotoxin and innate Immunity Society)
  • Int'l Joint Research
• [Remarks] 免疫不全を伴うプロテアソーム関連自己炎症症候群（遺伝性の炎症性疾患）の発見とそのモデルマウスの樹立
  • URL: https://www.wakayama-med.ac.jp/intro/press/r3/211207/07_menekifuzen.html
• [Remarks] COPA症候群（遺伝性の炎症性疾患）の病態を反映する新規モデルマウスを樹立
  • URL: https://www.wakayama-med.ac.jp/intro/press/r3/210628/28_seitai.html