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1. Biallelic human SHARPIN loss of function induces autoinflammation and immunodeficiency

Author

Oda, Hirotsugu, Manthiram, Kalpana, Chavan, Pallavi Pimpale, Rieser, Eva, Veli, Önay, Kaya, Öykü, Rauch, Charles, Nakabo, Shuichiro, Kuehn, Hye Sun, Swart, Mariël, Wang, Yanli, Çelik, Nisa Ilgim, Molitor, Anne, Ziaee, Vahid, Movahedi, Nasim, Shahrooei, Mohammad, Parvaneh, Nima, Alipour-olyei, Nasrin, Carapito, Raphael, Xu, Qin, Preite, Silvia, Beck, David B., Chae, Jae Jin, Nehrebecky, Michele, Ombrello, Amanda K., Hoffmann, Patrycja, Romeo, Tina, Deuitch, Natalie T., Matthíasardóttir, Brynja, Mullikin, James, Komarow, Hirsh, Stoddard, Jennifer, Niemela, Julie, Dobbs, Kerry, Sweeney, Colin L., Anderton, Holly, Lawlor, Kate E., Yoshitomi, Hiroyuki, Yang, Dan, Boehm, Manfred, Davis, Jeremy, Mudd, Pamela, Randazzo, Davide, Tsai, Wanxia Li, Gadina, Massimo, Kaplan, Mariana J., Toguchida, Junya, Mayer, Christian T., Rosenzweig, Sergio D., Notarangelo, Luigi D., Iwai, Kazuhiro, Silke, John, Schwartzberg, Pamela L., Boisson, Bertrand, Casanova, Jean-Laurent, Bahram, Seiamak, Rao, Anand Prahalad, Peltzer, Nieves, Walczak, Henning, Lalaoui, Najoua, Aksentijevich, Ivona, and Kastner, Daniel L.

Published
2024
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4. Perioperative Adriamycin plus ifosfamide vs. gemcitabine plus docetaxel for high-risk soft tissue sarcomas: randomised, phase II/III study JCOG1306

Author

Tanaka, Kazuhiro, Machida, Ryunosuke, Kawai, Akira, Nakayama, Robert, Tsukushi, Satoshi, Asanuma, Kunihiro, Matsumoto, Yoshihiro, Hiraga, Hiroaki, Hiraoka, Koji, Watanuki, Munenori, Yonemoto, Tsukasa, Abe, Satoshi, Katagiri, Hirohisa, Nishida, Yoshihiro, Nagano, Akihito, Suehara, Yoshiyuki, Kawashima, Hiroyuki, Kawano, Masanori, Morii, Takeshi, Hatano, Hiroshi, Toguchida, Junya, Okuma, Tomotake, Takeyama, Masanobu, Takenaka, Satoshi, Akisue, Toshihiro, Furuta, Taisuke, Emori, Makoto, Hiruma, Toru, Outani, Hidetatsu, Yamamoto, Tetsuji, Kataoka, Tomoko, Fukuda, Haruhiko, Ozaki, Toshifumi, and Iwamoto, Yukihide

Published
2022
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5. A de novo dominant-negative variant is associated with OTULIN-related autoinflammatory syndrome

Author

Takeda, Yukiko, primary, Ueki, Masahiro, additional, Matsuhiro, Junpei, additional, Walinda, Erik, additional, Tanaka, Takayuki, additional, Yamada, Masafumi, additional, Fujita, Hiroaki, additional, Takezaki, Shunichiro, additional, Kobayashi, Ichiro, additional, Tamaki, Sakura, additional, Nagata, Sanae, additional, Miyake, Noriko, additional, Matsumoto, Naomichi, additional, Osawa, Mitsujiro, additional, Yasumi, Takahiro, additional, Heike, Toshio, additional, Ohtake, Fumiaki, additional, Saito, Megumu K., additional, Toguchida, Junya, additional, Takita, Junko, additional, Ariga, Tadashi, additional, and Iwai, Kazuhiro, additional

Published
2024
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15. BMP-SMAD-ID promotes reprogramming to pluripotency by inhibiting p16/INK4A-dependent senescence

Author

Hayashi, Yohei, Hsiao, Edward C, Sami, Salma, Lancero, Mariselle, Schlieve, Christopher R, Nguyen, Trieu, Yano, Koyori, Nagahashi, Ayako, Ikeya, Makoto, Matsumoto, Yoshihisa, Nishimura, Ken, Fukuda, Aya, Hisatake, Koji, Tomoda, Kiichiro, Asaka, Isao, Toguchida, Junya, Conklin, Bruce R, and Yamanaka, Shinya

Subjects
Stem Cell Research, Stem Cell Research - Induced Pluripotent Stem Cell - Human, Stem Cell Research - Induced Pluripotent Stem Cell, 2.1 Biological and endogenous factors, Aetiology, Underpinning research, 1.1 Normal biological development and functioning, Activin Receptors, Type I, Adolescent, Adult, Animals, Bone Morphogenetic Proteins, Cell Line, Cellular Reprogramming, Cellular Senescence, Child, Cyclin-Dependent Kinase Inhibitor p16, Female, Fibroblasts, Humans, Induced Pluripotent Stem Cells, Male, Mice, Transgenic, Middle Aged, Mutation, Myositis Ossificans, Signal Transduction, Smad Proteins, reprogramming, pluripotency, BMP, senescence, FOP
Abstract

Fibrodysplasia ossificans progressiva (FOP) patients carry a missense mutation in ACVR1 [617G > A (R206H)] that leads to hyperactivation of BMP-SMAD signaling. Contrary to a previous study, here we show that FOP fibroblasts showed an increased efficiency of induced pluripotent stem cell (iPSC) generation. This positive effect was attenuated by inhibitors of BMP-SMAD signaling (Dorsomorphin or LDN1931890) or transducing inhibitory SMADs (SMAD6 or SMAD7). In normal fibroblasts, the efficiency of iPSC generation was enhanced by transducing mutant ACVR1 (617G > A) or SMAD1 or adding BMP4 protein at early times during the reprogramming. In contrast, adding BMP4 at later times decreased iPSC generation. ID genes, transcriptional targets of BMP-SMAD signaling, were critical for iPSC generation. The BMP-SMAD-ID signaling axis suppressed p16/INK4A-mediated cell senescence, a major barrier to reprogramming. These results using patient cells carrying the ACVR1 R206H mutation reveal how cellular signaling and gene expression change during the reprogramming processes.

Published
2016

16. Recapitulating the human segmentation clock with pluripotent stem cells

Author

Matsuda, Mitsuhiro, Yamanaka, Yoshihiro, Uemura, Maya, Osawa, Mitsujiro, Saito, Megumu K., Nagahashi, Ayako, Nishio, Megumi, Guo, Long, Ikegawa, Shiro, Sakurai, Satoko, Kihara, Shunsuke, Maurissen, Thomas L., Nakamura, Michiko, Matsumoto, Tomoko, Yoshitomi, Hiroyuki, Ikeya, Makoto, Kawakami, Noriaki, Yamamoto, Takuya, Woltjen, Knut, Ebisuya, Miki, Toguchida, Junya, and Alev, Cantas

Published
2020
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18. Induced pluripotent stem cells from patients with human fibrodysplasia ossificans progressiva show increased mineralization and cartilage formation

Author

Matsumoto, Yoshihisa, Hayashi, Yohei, Schlieve, Christopher R, Ikeya, Makoto, Kim, Hannah, Nguyen, Trieu D, Sami, Salma, Baba, Shiro, Barruet, Emilie, Nasu, Akira, Asaka, Isao, Otsuka, Takanobu, Yamanaka, Shinya, Conklin, Bruce R, Toguchida, Junya, and Hsiao, Edward C

Abstract

Abstract Background Abnormal activation of endochondral bone formation in soft tissues causes significant medical diseases associated with disability and pain. Hyperactive mutations in the bone morphogenetic protein (BMP) type 1 receptor ACVR1 lead to fibrodysplasia ossificans progressiva (FOP), a rare genetic disorder characterized by progressive ossification in soft tissues. However, the specific cellular mechanisms are unclear. In addition, the difficulty obtaining tissue samples from FOP patients and the limitations in mouse models of FOP hamper our ability to dissect the pathogenesis of FOP. Methods To address these challenges and develop a “disease model in a dish”, we created human induced pluripotent stem cells (iPS cells) derived from normal and FOP dermal fibroblasts by two separate methods, retroviral integration or integration-free episomal vectors. We tested if the ability to contribute to different steps of endochondral bone formation was different in FOP vs. control iPS cells. Results Remarkably, FOP iPS cells showed increased mineralization and enhanced chondrogenesis in vitro. The mineralization phenotypes could be suppressed with a small-molecule inhibitor of BMP signaling, DMH1. Our results indicate that the FOP ACVR1 R206H mutation favors chondrogenesis and increases mineral deposition in vitro. Conclusions Our findings establish a FOP disease cell model for in vitro experimentation and provide a proof-of-concept for using human iPS cell models to understand human skeletal disorders.

Published
2013

22. Heterozygous mutations in the straitjacket region of the latency-associated peptide domain of TGFB2 cause Camurati–Engelmann disease type II

Author

Wang, Zheng, Kometani, Mitsuhiro, Zeitlin, Leonid, Wilnai, Yael, Kinoshita, Akira, Yoshiura, Koh-ichiro, Ninomiya, Hiroko, Imamura, Takeshi, Guo, Long, Xue, Jingyi, Yan, Li, Ohashi, Hirofumi, Pretemer, Yann, Kawai, Shunsuke, Shiina, Masaaki, Ogata, Kazuhiro, Cohn, Daniel H., Matsumoto, Naomichi, Nishimura, Gen, Toguchida, Junya, Miyake, Noriko, and Ikegawa, Shiro

Abstract

Camurati–Engelmann disease (CED) is an autosomal dominant bone dysplasia characterized by progressive hyperostosis of the skull base and diaphyses of the long bones. CED is further divided into two subtypes, CED1 and CED2, according to the presence or absence of TGFB1mutations, respectively. In this study, we used exome sequencing to investigate the genetic cause of CED2 in three pedigrees and identified two de novo heterozygous mutations in TGFB2among the three patients. Both mutations were located in the region of the gene encoding the straitjacket subdomain of the latency-associated peptide (LAP) of pro-TGF-β2. Structural simulations of the mutant LAPs suggested that the mutations could cause significant conformational changes and lead to a reduction in TGF-β2 inactivation. An activity assay confirmed a significant increase in TGF-β2/SMAD signaling. In vitro osteogenic differentiation experiment using iPS cells from one of the CED2 patients showed significantly enhanced ossification, suggesting that the pathogenic mechanism of CED2 is increased activation of TGF-β2 by loss-of-function of the LAP. These results, in combination with the difference in hyperostosis patterns between CED1 and CED2, suggest distinct functions between TGFB1and TGFB2in human skeletal development and homeostasis.

Published
2024
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23. Biallelic human SHARPINloss of function induces autoinflammation and immunodeficiency

Author

Oda, Hirotsugu, Manthiram, Kalpana, Chavan, Pallavi Pimpale, Rieser, Eva, Veli, Önay, Kaya, Öykü, Rauch, Charles, Nakabo, Shuichiro, Kuehn, Hye Sun, Swart, Mariël, Wang, Yanli, Çelik, Nisa Ilgim, Molitor, Anne, Ziaee, Vahid, Movahedi, Nasim, Shahrooei, Mohammad, Parvaneh, Nima, Alipour-olyei, Nasrin, Carapito, Raphael, Xu, Qin, Preite, Silvia, Beck, David B., Chae, Jae Jin, Nehrebecky, Michele, Ombrello, Amanda K., Hoffmann, Patrycja, Romeo, Tina, Deuitch, Natalie T., Matthíasardóttir, Brynja, Mullikin, James, Komarow, Hirsh, Stoddard, Jennifer, Niemela, Julie, Dobbs, Kerry, Sweeney, Colin L., Anderton, Holly, Lawlor, Kate E., Yoshitomi, Hiroyuki, Yang, Dan, Boehm, Manfred, Davis, Jeremy, Mudd, Pamela, Randazzo, Davide, Tsai, Wanxia Li, Gadina, Massimo, Kaplan, Mariana J., Toguchida, Junya, Mayer, Christian T., Rosenzweig, Sergio D., Notarangelo, Luigi D., Iwai, Kazuhiro, Silke, John, Schwartzberg, Pamela L., Boisson, Bertrand, Casanova, Jean-Laurent, Bahram, Seiamak, Rao, Anand Prahalad, Peltzer, Nieves, Walczak, Henning, Lalaoui, Najoua, Aksentijevich, Ivona, and Kastner, Daniel L.

Abstract

The linear ubiquitin assembly complex (LUBAC) consists of HOIP, HOIL-1 and SHARPIN and is essential for proper immune responses. Individuals with HOIP and HOIL-1 deficiencies present with severe immunodeficiency, autoinflammation and glycogen storage disease. In mice, the loss of Sharpinleads to severe dermatitis due to excessive keratinocyte cell death. Here, we report two individuals with SHARPIN deficiency who manifest autoinflammatory symptoms but unexpectedly no dermatological problems. Fibroblasts and B cells from these individuals showed attenuated canonical NF-κB responses and a propensity for cell death mediated by TNF superfamily members. Both SHARPIN-deficient and HOIP-deficient individuals showed a substantial reduction of secondary lymphoid germinal center B cell development. Treatment of one SHARPIN-deficient individual with anti-TNF therapies led to complete clinical and transcriptomic resolution of autoinflammation. These findings underscore the critical function of the LUBAC as a gatekeeper for cell death-mediated immune dysregulation in humans.

Published
2024
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25. Supplementary Figure S5 from IGF2 Preserves Osteosarcoma Cell Survival by Creating an Autophagic State of Dormancy That Protects Cells against Chemotherapeutic Stress

Author

Shimizu, Takatsune, primary, Sugihara, Eiji, primary, Yamaguchi-Iwai, Sayaka, primary, Tamaki, Sakura, primary, Koyama, Yuko, primary, Kamel, Walied, primary, Ueki, Arisa, primary, Ishikawa, Tomoki, primary, Chiyoda, Tatsuyuki, primary, Osuka, Satoru, primary, Onishi, Nobuyuki, primary, Ikeda, Hiroko, primary, Kamei, Junzo, primary, Matsuo, Koichi, primary, Fukuchi, Yumi, primary, Nagai, Toshihiro, primary, Toguchida, Junya, primary, Toyama, Yoshiaki, primary, Muto, Akihiro, primary, and Saya, Hideyuki, primary

Published
2023
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26. Supplementary movie S1 from IGF2 Preserves Osteosarcoma Cell Survival by Creating an Autophagic State of Dormancy That Protects Cells against Chemotherapeutic Stress

Author

Shimizu, Takatsune, primary, Sugihara, Eiji, primary, Yamaguchi-Iwai, Sayaka, primary, Tamaki, Sakura, primary, Koyama, Yuko, primary, Kamel, Walied, primary, Ueki, Arisa, primary, Ishikawa, Tomoki, primary, Chiyoda, Tatsuyuki, primary, Osuka, Satoru, primary, Onishi, Nobuyuki, primary, Ikeda, Hiroko, primary, Kamei, Junzo, primary, Matsuo, Koichi, primary, Fukuchi, Yumi, primary, Nagai, Toshihiro, primary, Toguchida, Junya, primary, Toyama, Yoshiaki, primary, Muto, Akihiro, primary, and Saya, Hideyuki, primary

Published
2023
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27. Data from IGF2 Preserves Osteosarcoma Cell Survival by Creating an Autophagic State of Dormancy That Protects Cells against Chemotherapeutic Stress

Author

Shimizu, Takatsune, primary, Sugihara, Eiji, primary, Yamaguchi-Iwai, Sayaka, primary, Tamaki, Sakura, primary, Koyama, Yuko, primary, Kamel, Walied, primary, Ueki, Arisa, primary, Ishikawa, Tomoki, primary, Chiyoda, Tatsuyuki, primary, Osuka, Satoru, primary, Onishi, Nobuyuki, primary, Ikeda, Hiroko, primary, Kamei, Junzo, primary, Matsuo, Koichi, primary, Fukuchi, Yumi, primary, Nagai, Toshihiro, primary, Toguchida, Junya, primary, Toyama, Yoshiaki, primary, Muto, Akihiro, primary, and Saya, Hideyuki, primary

Published
2023
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30. Development of an osteosarcoma model withMYCNamplification andTP53mutation in hiPS cell‐derived neural crest cells

Author

Mukae, Kyosuke, primary, Takenobu, Hisanori, additional, Endo, Yuki, additional, Haruta, Masayuki, additional, Shi, Tianyuan, additional, Satoh, Shunpei, additional, Ohira, Miki, additional, Funato, Michinori, additional, Toguchida, Junya, additional, Osafune, Kenji, additional, Nakahata, Tatsutoshi, additional, Kanda, Hiroaki, additional, and Kamijo, Takehiko, additional

Published
2023
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34. Activin-A enhances mTOR signaling to promote aberrant chondrogenesis in fibrodysplasia ossificans progressiva

Author

Hino, Kyosuke, Horigome, Kazuhiko, Nishio, Megumi, Komura, Shingo, Nagata, Sanae, Zhao, Chengzhu, Jin, Yonghui, Kawakami, Koichi, Yamada, Yasuhiro, Ohta, Akira, Toguchida, Junya, and Ikeya, Makoto

Subjects
Rare diseases -- Physiological aspects, Genetic disorders -- Physiological aspects, Rapamycin -- Health aspects, Health care industry
Abstract

Fibrodysplasia ossificans progressiva (FOP) is a rare and intractable disease characterized by extraskeletal bone formation through endochondral ossification. Patients with FOP harbor point mutations in ACVR1, a type I receptor for BMPs. Although mutated ACVR1 (FOP-ACVR1) has been shown to render hyperactivity in BMP signaling, we and others have uncovered a mechanism by which FOP-ACVR1 mistransduces BMP signaling in response to Activin -A, a molecule that normally transduces TGF-[beta] signaling. Although Activin-A evokes enhanced chondrogenesis in vitro and heterotopic ossification (HO) in vivo, the underlying mechanisms have yet to be revealed. To this end, we developed a high -throughput screening (HTS) system using FOP patient-derived induced pluripotent stem cells (FOP-iPSCs) to identify pivotal pathways in enhanced chondrogenesis that are initiated by Activin-A. In a screen of 6,809 small-molecule compounds, we identified mTOR signaling as a critical pathway for the aberrant chondrogenesis of mesenchymal stromal cells derived from FOP-iPSCs (FOP-iMSCs). Two different HO mouse models, an FOP model mouse expressing FOP-ACVR1 and an FOP-iPSC-based HO model mouse, revealed critical roles for mTOR signaling in vivo. Moreover, we identified ENPP2, an enzyme that generates lysophosphatidic acid, as a linker of FOP-ACVR1 and mTOR signaling in chondrogenesis. These results uncovered the crucial role of the Activin-A/FOP-ACVR1/ ENPP2/mTOR axis in FOP pathogenesis., Introduction Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disease characterized by extraskeletal bone formation in soft tissue, where bone normally does not exist. Ectopic bones are formed through endochondral [...]

Published
2017
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35. Human LUBAC deficiency leads to autoinflammation and immunodeficiency by dysregulation in TNF-mediated cell death

Author

Oda, Hirotsugu, primary, Manthiram, Kalpana, additional, Chavan, Pallavi Pimpale, additional, Nakabo, Shuichiro, additional, Kuehn, Hye Sun, additional, Beck, David B., additional, Chae, Jae Jin, additional, Nehrebecky, Michele, additional, Ombrello, Amanda K., additional, Romeo, Tina, additional, Deuitch, Natalie, additional, Matthíasardóttir, Brynja, additional, Mullikin, Jim, additional, Stoddard, Jennifer, additional, Niemela, Julie, additional, Anderton, Holly, additional, Lawlor, Kate E., additional, Yoshitomi, Hiroyuki, additional, Yang, Dan, additional, Boehm, Manfred, additional, Davis, Jeremy, additional, Mudd, Pamela, additional, Randazzo, Davide, additional, Tsai, Wanxia Li, additional, Gadina, Massimo, additional, Kaplan, Mariana J., additional, Toguchida, Junya, additional, Mayer, Christian, additional, Rosenzweig, Sergio D., additional, Iwai, Kazuhiro, additional, Silke, John, additional, Boisson, Bertrand, additional, Casanova, Jean-Laurent, additional, Rao, Anand, additional, Lalaoui, Najoua, additional, Aksentijevich, Ivona, additional, and Kastner, Daniel L., additional

Published
2022
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39. Clinical Outcomes of Patients With Osteosarcoma Experiencing Relapse or Progression: A Single-institute Experience

Author

Umeda, Katsutsugu, primary, Sakamoto, Akio, additional, Noguchi, Takashi, additional, Uchihara, Yoshinori, additional, Kobushi, Hirokazu, additional, Akazawa, Ryo, additional, Ogata, Hideto, additional, Saida, Satoshi, additional, Kato, Itaru, additional, Hiramatsu, Hidefumi, additional, Uto, Megumi, additional, Mizowaki, Takashi, additional, Haga, Hironori, additional, Date, Hiroshi, additional, Okamoto, Takeshi, additional, Watanabe, Kenichiro, additional, Adachi, Souichi, additional, Toguchida, Junya, additional, Matsuda, Shuichi, additional, and Takita, Junko, additional

Published
2022
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40. 術前経カテーテル動脈塞栓術後に下腹部正中切開と殿部後方到達法を併用し完全摘除した骨盤内単中心性Castleman病の1例

Author

SUZUKI, Ryosuke, GOTO, Takayuki, BANNO, Haruka, FUCHIGAMI, Yasushi, HIDA, Takuya, FUJIWARA, Maki, YOSHINO, Takayuki, KITA, Yuki, SAWADA, Atsuro, AKAMATSU, Shusuke, SAITO, Ryoichi, KOBAYASHI, Takashi, YAMAZAKI, Toshinari, INOUE, Takahiro, SHIMIZU, Hironori, KURATA, Mariyo, MAEDA, Hirona, OKAMOTO, Takeshi, TOGUCHIDA, Junya, and OGAWA, Osamu

Subjects
Posterior approach, Castleman disease, 494.9
Abstract

A 22-year-old woman was referred to our hospital for further examination of an incidentally discovered hypervascular pelvic tumor with a maximum diameter of 10 cm. Although Castleman disease was suspected based on the imaging findings and pathologic findings of the needle biopsy, a definitive diagnosis was not made. Preoperative transcatheter arterial embolization was performed to decrease intraoperative bleeding, and tumor resection was performed on the following day. As for posterior approach prior to anterior approach, the patient was placed in a prone position, and the dorsal aspect of tumor was approached through the dissection of gluteal muscles. Then, dilated branches of the internal iliac vein was found on the tumor capsule, which were safely ligated under direct vision with favorable visual field. Then, the patient was placed in a supine position, the tumor was completely resected by anterior approach without transfusion. Histopathological diagnosis was Castleman disease hyaline vascular type. The patient was discharged without complication and has been free from recurrence for 6 months after surgery.

Published
2021

42. Development of an osteosarcoma model with MYCN amplification and TP53 mutation in hiPS cell‐derived neural crest cells.

Author

Mukae, Kyosuke, Takenobu, Hisanori, Endo, Yuki, Haruta, Masayuki, Shi, Tianyuan, Satoh, Shunpei, Ohira, Miki, Funato, Michinori, Toguchida, Junya, Osafune, Kenji, Nakahata, Tatsutoshi, Kanda, Hiroaki, and Kamijo, Takehiko

Abstract

Mesenchymal stem cell‐ or osteoblast‐derived osteosarcoma is the most common malignant bone tumor. Its highly metastatic malignant phenotypes, which are often associated with a poor prognosis, have been correlated with the modulation of TP53‐ and cell‐cycle‐related pathways. MYC, which regulates the transcription of cell‐cycle modulating genes, is used as a representative prognostic marker for osteosarcoma. Another member of the MYC oncoprotein family, MYCN, is highly expressed in a subset of osteosarcoma, however its roles in osteosarcoma have not been fully elucidated. Here, we attempted to create an in vitro tumorigenesis model using hiPSC‐derived neural crest cells, which are precursors of mesenchymal stem cells, by overexpressing MYCN on a heterozygous TP53 hotspot mutation (c.733G>A; p.G245S) background. MYCN‐expressing TP53 mutated transformed clones were isolated by soft agar colony formation, and administered subcutaneously into the periadrenal adipose tissue of immunodeficient mice, resulting in the development of chondroblastic osteosarcoma. MYCN suppression decreased the proliferation of MYCN‐induced osteosarcoma cells, suggesting MYCN as a potential target for a subset of osteosarcoma treatment. Further, comprehensive analysis of gene expression and exome sequencing of MYCN‐induced clones indicated osteosarcoma‐specific molecular features, such as the activation of TGF‐β signaling and DNA copy number amplification of GLI1. The model of MYCN‐expressing chondroblastic osteosarcoma was developed from hiPSC‐derived neural crest cells, providing a useful tool for the development of new tumor models using hiPSC‐derived progenitor cells with gene modifications and in vitro transformation. [ABSTRACT FROM AUTHOR]

Published
2023
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43. Clinical Outcomes of Patients With Osteosarcoma Experiencing Relapse or Progression: A Single-institute Experience.

Author

Umeda, Katsutsugu, Sakamoto, Akio, Noguchi, Takashi, Uchihara, Yoshinori, Kobushi, Hirokazu, Akazawa, Ryo, Ogata, Hideto, Saida, Satoshi, Kato, Itaru, Hiramatsu, Hidefumi, Uto, Megumi, Mizowaki, Takashi, Haga, Hironori, Date, Hiroshi, Okamoto, Takeshi, Watanabe, Kenichiro, Adachi, Souichi, Toguchida, Junya, Matsuda, Shuichi, and Takita, Junko

Published
2023
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45. Prospective comparison of various radiological response criteria and pathological response to preoperative chemotherapy and survival in operable high-grade soft tissue sarcomas in the Japan Clinical Oncology Group study JCOG0304

Author

Tanaka, Kazuhiro, Ogawa, Gakuto, Mizusawa, Junki, Naka, Norifumi, Kawai, Akira, Takahashi, Mitsuru, Hiruma, Toru, Matsumoto, Yoshihiro, Tsuchiya, Hiroyuki, Nakayama, Robert, Hatano, Hiroshi, Emori, Makoto, Hosaka, Masami, Yoshida, Yukihiro, Toguchida, Junya, Abe, Satoshi, Asanuma, Kunihiro, Yokoyama, Ryohei, Hiraga, Hiroaki, Yonemoto, Tsukasa, Morii, Takeshi, Matsumoto, Seiichi, Nagano, Akihito, Yoshikawa, Hideki, Fukuda, Haruhiko, Ozaki, Toshifumi, and Iwamoto, Yukihide

Published
2018
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47. Identification of Disease Gene for Camurati-Engelmann Disease, Type II

Author

Wang, Zheng, primary, Xue, Jingyi, additional, Nishimura, Gen, additional, Kometani, Mitsuhiro, additional, Wilnai, Yael, additional, Zeitlin, Leonid, additional, Kinoshita, Akira, additional, Yoshiura, Koh-ichiro, additional, Imamura, Takeshi, additional, Ohashi, Hirofumi, additional, Kawai, Shunsuke, additional, Shiina, Masaaki, additional, Ogata, Kazuhiro, additional, Miyake, Noriko, additional, Toguchida, Junya, additional, Superti-Furga, Andrea, additional, Matsumoto, Naomichi, additional, and Ikegawa, Shiro, additional

Published
2022
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