Search

Your search keyword '"Onodera, Masafumi"' showing total 325 results
Start Over Author "Onodera, Masafumi" Publication Type Academic Journals
325 results on '"Onodera, Masafumi"'

4. Myelodysplasia after clonal hematopoiesis with APOBEC3-mediated CYBB inactivation in retroviral gene therapy for X-CGD

Author

Uchiyama, Toru, Kawai, Toshinao, Nakabayashi, Kazuhiko, Nakazawa, Yumiko, Goto, Fumihiro, Okamura, Kohji, Nishimura, Toyoki, Kato, Koji, Watanabe, Nobuyuki, Miura, Akane, Yasuda, Toru, Ando, Yukiko, Minegishi, Tomoko, Edasawa, Kaori, Shimura, Marika, Akiba, Yumi, Sato-Otsubo, Aiko, Mizukami, Tomoyuki, Kato, Motohiro, Akashi, Koichi, Nunoi, Hiroyuki, and Onodera, Masafumi

Published
2023
Full Text
View/download PDF

12. A prospective study of allogeneic transplantation from unrelated donors for chronic granulomatous disease with target busulfan-based reduced-intensity conditioning

Author

Osumi, Tomoo, Tomizawa, Daisuke, Kawai, Toshinao, Sako, Mayumi, Inoue, Eisuke, Takimoto, Tetsuya, Tamura, Eiichiro, Uchiyama, Toru, Imadome, Ken-Ichi, Taniguchi, Maki, Shirai, Ryota, Yoshida, Masanori, Ando, Rie, Tsumura, Yusuke, Fuji, Hiroshi, Matsumoto, Kana, Shioda, Yoko, Kiyotani, Chikako, Terashima, Keita, Onodera, Masafumi, Matsumoto, Kimikazu, and Kato, Motohiro

Published
2019
Full Text
View/download PDF

15. Haploinsufficiency of A20 caused by a novel nonsense variant or entire deletion of TNFAIP3 is clinically distinct from Behçet’s disease

Author

Tsuchida, Naomi, Kirino, Yohei, Soejima, Yutaro, Onodera, Masafumi, Arai, Katsuhiro, Tamura, Eiichiro, Ishikawa, Takashi, Kawai, Toshinao, Uchiyama, Toru, Nomura, Shigeru, Kobayashi, Daisuke, Taguri, Masataka, Mitsuhashi, Satomi, Mizuguchi, Takeshi, Takata, Atsushi, Miyake, Noriko, Nakajima, Hideaki, Miyatake, Satoko, and Matsumoto, Naomichi

Published
2019
Full Text
View/download PDF

16. Ferroptosis model system by the re-expression of BACH1.

Author

Irikura, Riko, Nishizawa, Hironari, Nakajima, Kazuma, Yamanaka, Mie, Chen, Guan, Tanaka, Kozo, Onodera, Masafumi, Matsumoto, Mitsuyo, and Igarashi, Kazuhiko

Subjects
TRANSCRIPTION factors, IRON metabolism, GLUTAMATE transporters, ANIMAL species, GLUTATHIONE peroxidase, IRON
Abstract

Ferroptosis is a regulated cell death induced by iron-dependent lipid peroxidation. The heme-responsive transcription factor BTB and CNC homology 1 (BACH1) promotes ferroptosis by repressing the transcription of genes involved in glutathione (GSH) synthesis and intracellular labile iron metabolism, which are key regulatory pathways in ferroptosis. We found that BACH1 re-expression in Bach1 −/− immortalized mouse embryonic fibroblasts (iMEFs) can induce ferroptosis upon 2-mercaptoethanol removal, without any ferroptosis inducers. In these iMEFs, GSH synthesis was reduced, and intracellular labile iron levels were increased upon BACH1 re-expression. We used this system to investigate whether the major ferroptosis regulators glutathione peroxidase 4 (Gpx4) and apoptosis-inducing factor mitochondria-associated 2 (Aifm2), the gene for ferroptosis suppressor protein 1, are target genes of BACH1. Neither Gpx4 nor Aifm2 was regulated by BACH1 in the iMEFs. However, we found that BACH1 represses AIFM2 transcription in human pancreatic cancer cells. These results suggest that the ferroptosis regulators targeted by BACH1 may vary across different cell types and animal species. Furthermore, we confirmed that the ferroptosis induced by BACH1 re-expression exhibited a propagating effect. BACH1 re-expression represents a new strategy for inducing ferroptosis after GPX4 or system Xc− suppression and is expected to contribute to future ferroptosis research. [ABSTRACT FROM AUTHOR]

Published
2023
Full Text
View/download PDF

19. Safety and efficacy of elapegademase in patients with adenosine deaminase deficiency: A multicenter, open‐label, single‐arm, phase 3, and postmarketing clinical study.

Author

Onodera, Masafumi, Uchiyama, Toru, Ariga, Tadashi, Yamada, Masafumi, Miyamura, Takako, Arizono, Hironori, and Morio, Tomohiro

Subjects
*ADENOSINE deaminase, *SEVERE combined immunodeficiency, *IMMUNOGLOBULIN M, *IMMUNOGLOBULIN A, *CYTOMEGALOVIRUS diseases
Abstract

Introduction: Adenosine deaminase (ADA) deficiency is an ultrarare inherited purine metabolism disorder characterized by severe combined immunodeficiency. Elapegademase‐lvlr is a new pegylated recombinant bovine ADA used in enzyme‐replacement therapy (ERT) for ADA deficiency. Therefore, replacement with the new drug may eliminate the infectious risks associated with the currently used bovine intestinal‐derived product, pegademase. Methods: We conducted a multicenter, single‐arm, open‐label, phase 3, and postmarketing clinical study of elapegademase for patients with ADA deficiency. The following biochemical markers were monitored to determine an appropriate dose of elapegademase: the trough deoxyadenosine nucleotide (dAXP) level ≤0.02 μmol/mL in erythrocytes or whole blood and the trough serum ADA activity ≥1100 U/L (equivalent to plasma levels ≥15 μmol/h/mL) indicated sufficient enzyme activity and detoxification as efficacy endpoints and monitored adverse events during the study as safety endpoints. Results: A total of four patients (aged 0–25 years) were enrolled. One infant patient died of pneumonia caused by cytomegalovirus infection whereas the other three completed the study and have been observed in the study period over 3 years. The infant patient had received elapegademase at 0.4 mg/kg/week until decease and the others received elapegademase at maximum doses of 0.3 mg/kg/week for 164–169 weeks. As a result, all four patients achieved undetectable levels of dAXPs together with sufficient enzyme activity, increased T and B cell numbers, and slightly elevated and maintained IgM and IgA immunoglobulin levels. Serious adverse events occurred in three patients, all of which were assessed as unrelated to elapegademase. Conclusions: This study showed that elapegademase had comparable safety and efficacy to pegademase as ERT for ADA deficiency by demonstrating stable maintenance of sufficient ADA activity and lowering dAXP to undetectable levels, while no drug‐related adverse events were reported (Trial registration: JapicCTI‐163204). [ABSTRACT FROM AUTHOR]

Published
2023
Full Text
View/download PDF

22. Outcomes in Two Japanese Adenosine Deaminase-Deficiency Patients Treated by Stem Cell Gene Therapy with No Cytoreductive Conditioning

Author

Otsu, Makoto, Yamada, Masafumi, Nakajima, Satoru, Kida, Miyuki, Maeyama, Yoshihiro, Hatano, Norikazu, Toita, Nariaki, Takezaki, Shunichiro, Okura, Yuka, Kobayashi, Ryoji, Matsumoto, Yoshinori, Tatsuzawa, Osamu, Tsuchida, Fumiko, Kato, Shunichi, Kitagawa, Masanari, Mineno, Junichi, Hershfield, Michael S., Bali, Pawan, Candotti, Fabio, Onodera, Masafumi, Kawamura, Nobuaki, Sakiyama, Yukio, and Ariga, Tadashi

Published
2015
Full Text
View/download PDF

24. Identification of Tumor-Initiating Cells in a Highly Aggressive Brain Tumor Using Promoter Activity of Nucleostemin

Author

Tamase, Akira, Muraguchi, Teruyuki, Naka, Kazuhito, Tanaka, Shingo, Kinoshita, Masashi, Hoshii, Takayuki, Ohmura, Masako, Shugo, Haruhiko, Ooshio, Takako, Nakada, Mitsutoshi, Sawamoto, Kazunobu, Onodera, Masafumi, Matsumoto, Kunio, Oshima, Masanobu, Asano, Masahide, Saya, Hideyuki, Okano, Hideyuki, Suda, Toshio, Hamada, Jun-Ichiro, Hirao, Atsushi, and Mak, Tak Wah

Published
2009
Full Text
View/download PDF

32. Prospective Study of Allogeneic Hematopoietic Stem Cell Transplantation with Post-Transplantation Cyclophosphamide and Antithymocyte Globulin from HLA-Mismatched Related Donors for Nonmalignant Diseases

Author

Osumi, Tomoo, Yoshimura, Satoshi, Sako, Mayumi, Uchiyama, Toru, Ishikawa, Takashi, Kawai, Toshinao, Inoue, Eisuke, Takimoto, Tetsuya, Takeuchi, Ichiro, Yamada, Masaki, Sakamoto, Kenichi, Yoshida, Kaoru, Kimura, Yui, Matsukawa, Yukihiro, Matsumoto, Kana, Imadome, Ken-Ichi, Arai, Katsuhiro, Deguchi, Takao, Imai, Kohsuke, Yuza, Yuki, Matsumoto, Kimikazu, Onodera, Masafumi, Kanegane, Hirokazu, Tomizawa, Daisuke, and Kato, Motohiro

Published
2020
Full Text
View/download PDF

44. Gain-of-function of mutated C-CBL tumour suppressor in myeloid neoplasms

Author

Sanada, Masashi, Suzuki, Takahiro, Shih, Lee-Yung, Otsu, Makoto, Kato, Motohiro, Yamazaki, Satoshi, Tamura, Azusa, Honda, Hiroaki, Sakata-Yanagimoto, Mamiko, Kumano, Keiki, Oda, Hideaki, Yamagata, Tetsuya, Takita, Junko, Gotoh, Noriko, Nakazaki, Kumi, Kawamata, Norihiko, Onodera, Masafumi, Nobuyoshi, Masaharu, Hayashi, Yasuhide, Harada, Hiroshi, Kurokawa, Mineo, Chiba, Shigeru, Mori, Hiraku, Ozawa, Keiya, Omine, Mitsuhiro, Hirai, Hisamaru, Nakauchi, Hiromitsu, Koeffler, Phillip H., and Ogawa, Seishi

Published
2009
Full Text
View/download PDF

46. Production of therapeutic iduronate‐2‐sulfatase enzyme with a novel single‐stranded RNA virus vector.

Author

Ohira, Mari, Kikuchi, Emika, Mizuta, Shiori, Yoshida, Naomi, Onodera, Masafumi, Nakanishi, Mahito, Okuyama, Torayuki, and Mashima, Ryuichi

Subjects
GREEN fluorescent protein, RNA viruses, SINGLE-stranded DNA, RNA, SENDAI virus, BASE pairs, LYSOSOMES
Abstract

The Sendai virus vector has received a lot of attention due to its broad tropism for mammalian cells. As a result of efforts for genetic studies based on a mutant virus, we can now express more than 10 genes of up to 13.5 kilo nucleotides in a single vector with high protein expression efficiency. To prove this benefit, we examined the efficacy of the novel ribonucleic acid (RNA) virus vector harboring the human iduronate‐2‐sulfatase (IDS) gene with 1,653 base pairs, a causative gene for mucopolysaccharidosis type II, also known as a disorder of lysosomal storage disorders. As expected, this novel RNA vector with the human IDS gene exhibited its marked expression as determined by the expression of enhanced green fluorescent protein and IDS enzyme activity. While these cells exhibited a normal growth rate, the BHK‐21 transformant cells stably expressing the human IDS gene persistently generated an active human IDS enzyme extracellularly. The human IDS protein produced failed to be incorporated into the lysosome when cells were pretreated with mannose‐6‐phosphate, demonstrating that this human IDS enzyme has potential for therapeutic use by cross‐correction. These results suggest that our novel RNA vector may be applicable for further clinical settings. [ABSTRACT FROM AUTHOR]

Published
2021
Full Text
View/download PDF

47. Bacillus Calmette-Guérin (BCG) Infections at High Frequency in Both AR-CGD and X-CGD Patients Following BCG Vaccination.

Author

Ishikawa, Takashi, Okai, Masashi, Mochizuki, Emi, Uchiyama, Toru, Onodera, Masafumi, and Kawai, Toshinao

Subjects
INFECTION prevention, CHRONIC disease treatment, GRANULOMA, IMMUNIZATION, GENETIC mutation, CHRONIC diseases, RETROSPECTIVE studies, MEDICAL protocols, BCG vaccines, SYMPTOMS, AGE factors in disease, FAMILY history (Medicine)
Abstract

Background Patients with chronic granulomatous disease (CGD) develop severe infections, including Bacillus Calmette-Guérin (BCG). Although the autosomal recessive CGD (AR-CGD) patients should hypothetically develop relatively fewer infections compared to the X-linked CGD (X-CGD) patients due to more residual reactive oxygen intermediates, the impacts of BCG vaccination on AR-CGD and X-CGD patients are unclear. Herein, we demonstrated the clinical features of BCG infections, treatments, and genetic factors in CGD patients after BCG vaccination under the Japanese immunization program. Methods We collected data retrospectively from 43 patients with CGD and assessed their history of initial infection, age at diagnosis of CGD, BCG vaccination history, clinical course, treatment for BCG infections, and genetic mutations associated with CGD. Results Fourteen CGD patients avoided BCG vaccination because of other preceding infections and family history. Of 29 patients with CGD who received BCG vaccination, 20 patients developed BCG infections. Although the age at onset of initial infection in X-CGD patients was significantly younger than that in AR-CGD patients (P  < .01), the onset and frequency of BCG infections were similar in X-CGD and AR-CGD patients. In X-CGD patients, BCG infections equally developed in the patients carrying missense, insertion, deletion, nonsense, and splice mutations of CYBB. All CGD patients with BCG infections were successfully treated with anti-tuberculous drugs. Conclusions Although X-CGD patients develop severe infections at a younger age than AR-CGD patients, our data suggested that BCG infections develop at high frequency in both AR-CGD and X-CGD patients, regardless of genotype and mutant forms. [ABSTRACT FROM AUTHOR]

Published
2021
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results