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18 results on '"Shimbo, Miki"'

5. Early loss of Scribble affects cortical development, interhemispheric connectivity and psychomotor activity.

Author

UCL - SSS/IONS/CEMO - Pôle Cellulaire et moléculaire, Ezan, Jerome, Moreau, Maité M, Mamo, Tamrat M, Shimbo, Miki, Decroo, Maureen, Richter, Melanie, Peyroutou, Ronan, Rachel, Rivka, Tissir, Fadel, de Anda, Froylan Calderon, Sans, Nathalie, Montcouquiol, Mireille, UCL - SSS/IONS/CEMO - Pôle Cellulaire et moléculaire, Ezan, Jerome, Moreau, Maité M, Mamo, Tamrat M, Shimbo, Miki, Decroo, Maureen, Richter, Melanie, Peyroutou, Ronan, Rachel, Rivka, Tissir, Fadel, de Anda, Froylan Calderon, Sans, Nathalie, and Montcouquiol, Mireille

Abstract

Neurodevelopmental disorders arise from combined defects in processes including cell proliferation, differentiation, migration and commissure formation. The evolutionarily conserved tumor-suppressor protein Scribble (Scrib) serves as a nexus to transduce signals for the establishment of apicobasal and planar cell polarity during these processes. Human SCRIB gene mutations are associated with neural tube defects and this gene is located in the minimal critical region deleted in the rare Verheij syndrome. In this study, we generated brain-specific conditional cKO mouse mutants and assessed the impact of the Scrib deletion on brain morphogenesis and behavior. We showed that embryonic deletion of Scrib in the telencephalon leads to cortical thickness reduction (microcephaly) and partial corpus callosum and hippocampal commissure agenesis. We correlated these phenotypes with a disruption in various developmental mechanisms of corticogenesis including neurogenesis, neuronal migration and axonal connectivity. Finally, we show that Scrib cKO mice have psychomotor deficits such as locomotor activity impairment and memory alterations. Altogether, our results show that Scrib is essential for early brain development due to its role in several developmental cellular mechanisms that could underlie some of the deficits observed in complex neurodevelopmental pathologies.

Published
2021

6. Selective neurodevelopmental and behavioral deficits in Scrib conditional knock-out mice recapitulate some phenotypes associated with the Verheij/8q23.4 deletion syndrome

Author

Ezan, Jerome, Moreau, Maité M., Mamo, Tamrat M., Shimbo, Miki, Decroo, Maureen, Richter, Melanie, Peyroutou, Ronan, Rachel, Rivka, Tissir, Fadel, Calderon de Anda, Froylan, Sans, Nathalie, and Montcouquiol, Mireille

Abstract

Neurodevelopmental disorders often arise from combined defects in processes including cell proliferation, differentiation, neuronal migration, axonal pathfinding and commissure formation. Cell polarity proteins serve as a nexus to transduce signals for the establishment of these essential processes. Scribble (Scrib) is an evolutionarily conserved polarity protein that is known to regulate the establishment of apicobasal and planar cell polarity. Mutations in the human SCRIB gene are associated with neural tube defects and this gene is located in the minimal critical region deleted in the rare Verheij/8q24.3 deletion syndrome. In the present study, we evaluated the contribution of Scrib to some of the neurological features found in patients with this syndrome, including microcephaly and corpus callosum agenesis. Using various brain-specific conditional mouse mutants and in utero electroporation experiments, we assessed the impact of the spatio-temporal selective Scrib deletion on brain morphogenesis and animal behavior. Our results showed that global embryonic deletion of Scrib in the telencephalon lead to a reduction of cortical thickness and an alteration of interhemispheric connectivity. In addition, we identified cell-autonomous effects of Scrib on neuronal migration, and we suggest a non-cell-autonomous effect in axonal guidance. Finally, comparative behavioral analysis showed that mice with Scrib invalidation have psychomotor deficits. Altogether, our mouse models recapitulate a number of the phenotypes associated with Verheij/8q24.3 deletion syndrome patients, supporting the possibility that Scrib contributes to this rare disease. Author Summary The mammalian brain, seat of cognitive and behavioral processing, is the result of numerous, complex but coordinated mechanisms of development such as cell proliferation, migration, neuritogenesis, synaptogenesis and neural network formation. Patients with disruptions in these fundamental processes will typically exhibit neurodevelopmental disorders. As such, the rare Verheij syndrome is a condition where patients display some neurological features such as microcephaly (dramatic reduction in brain cortical size) or agenesis of the corpus callosum (loss of the main commissure between hemispheres). The human gene SCRIB is included within a region, identified as 8q24.3, which is deleted in patients with this syndrome. In this paper, we generated mouse models to assess the consequences of selective genetic inactivation of Scrib on the brain architecture and function. From the neuroanatomical standpoint, we show that Scrib controls the formation of the cerebral cortex and the corpus callosum. This is correlated with behavioral deficits such as disrupted locomotion, learning and memory. Altogether, this work validates the association between Scrib loss and the brain-associated clinical features observed in individuals with Verheij/8q24.3 deletion syndrome.

Published
2019
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7. Early loss of Scribble affects cortical development and interhemispheric connectivity resulting in psychomotor dysregulation

Author

Ezan, Jerome, primary, Moreau, Maité M., additional, Mamo, Tamrat M., additional, Shimbo, Miki, additional, Decroo, Maureen, additional, Richter, Melanie, additional, Peyroutou, Ronan, additional, Rachel, Rivka, additional, Tissir, Fadel, additional, de Anda, Froylan Calderon, additional, Sans, Nathalie, additional, and Montcouquiol, Mireille, additional

Published
2019
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8. Long-term hindlimb unloading causes a preferential reduction of medullary thymic epithelial cells expressing autoimmune regulator (Aire)

Author

Horie, Kenta, primary, Kudo, Takashi, additional, Yoshinaga, Riko, additional, Akiyama, Nobuko, additional, Sasanuma, Hiroki, additional, Kobayashi, Tetsuya J., additional, Shimbo, Miki, additional, Jeon, Hyojung, additional, Miyao, Takahisa, additional, Miyauchi, Maki, additional, Shirakawa, Masaki, additional, Shiba, Dai, additional, Yoshida, Nobuaki, additional, Muratani, Masafumi, additional, Takahashi, Satoru, additional, and Akiyama, Taishin, additional

Published
2018
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10. Incomplete clearance of apoptotic cells by core 1-derived O-glycan-deficient resident peritoneal macrophages

Author

Wakui, Hiromasa, primary, Fuseya, Sayaka, additional, Suzuki, Riku, additional, Shimbo, Miki, additional, Okada, Risa, additional, Hamada, Mitchito, additional, Kuno, Akihiro, additional, Hagiwara, Kozue, additional, Sato, Takashi, additional, Narimatsu, Hisashi, additional, Kudo, Takashi, additional, and Takahashi, Satoru, additional

Published
2018
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11. Postnatal lethality and chondrodysplasia in mice lacking both chondroitin sulfate N-acetylgalactosaminyltransferase-1 and -2

Author

Shimbo, Miki, primary, Suzuki, Riku, additional, Fuseya, Sayaka, additional, Sato, Takashi, additional, Kiyohara, Katsue, additional, Hagiwara, Kozue, additional, Okada, Risa, additional, Wakui, Hiromasa, additional, Tsunakawa, Yuki, additional, Watanabe, Hideto, additional, Kimata, Koji, additional, Narimatsu, Hisashi, additional, Kudo, Takashi, additional, and Takahashi, Satoru, additional

Published
2017
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12. Development of new experimental platform ‘MARS’—Multiple Artificial-gravity Research System—to elucidate the impacts of micro/partial gravity on mice

Author

Shiba, Dai, primary, Mizuno, Hiroyasu, additional, Yumoto, Akane, additional, Shimomura, Michihiko, additional, Kobayashi, Hiroe, additional, Morita, Hironobu, additional, Shimbo, Miki, additional, Hamada, Michito, additional, Kudo, Takashi, additional, Shinohara, Masahiro, additional, Asahara, Hiroshi, additional, Shirakawa, Masaki, additional, and Takahashi, Satoru, additional

Published
2017
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13. Ground-based assessment of JAXA mouse habitat cage unit by mouse phenotypic studies

Author

Shimbo, Miki, Kudo, Takashi, Hamada, Michito, Jeon, Hyojung, Imamura, Yuki, Asano, Keigo, Okada, Risa, Tsunakawa, Yuki, Mizuno, Seiya, Yagami, Kenichi, Ishikawa, Chihiro, Li, Haiyan, Shiga, Takashi, Ishida, Junji, Hamada, Juri, Murata, Kazuya, Ishimaru, Tomohiro, Hashimoto, Misuzu, Fukamizu, Akiyoshi, Yamane, Mutsumi, Ikawa, Masahito, Morita, Hironobu, Shinohara, Masahiro, Asahara, Hiroshi, Akiyama, Taishin, Akiyama, Nobuko, Sasanuma, Hiroki, Yoshida, Nobuaki, Zhou, Rui, Wang, Ying-Ying, Ito, Taito, Kokubu, Yuko, Noguchi, Takaaki, K., Ishimine, Hisako, Kurisaki, Akira, Shiba, Dai, Mizuno, Hiroyasu, Shirakawa, Masaki, Ito, Naoki, Takeda, Shin, Takahashi, Satoru, 新保, 未来, 工藤, 崇, 濱田, 理人, 今村, 優希, 浅野, 圭吾, 岡田, 理沙, 綱川, 祐貴, 水野, 聖哉, 八神, 健一, 石川, 千尋, 志賀, 隆, 石田, 純治, 濱田, 樹理, 村田, 知弥, 石丸, 友博, 橋本, 美涼, 深水, 昭吉, 山根, 睦, 伊川, 正人, 森田, 啓之, 篠原, 正浩, 浅原, 弘嗣, 秋山, 泰身, 秋山, 伸子, 笹沼, 寛樹, 吉田, 進昭, 伊藤, 泰斗, 國分, 優子, 野口, 隆明, 石嶺, 久子, 栗崎, 晃, 芝, 大, 水野, 浩靖, 白川, 正輝, 伊藤, 尚基, 武田, 伸一, 高橋, 智, Shimbo, Miki, Kudo, Takashi, Hamada, Michito, Jeon, Hyojung, Imamura, Yuki, Asano, Keigo, Okada, Risa, Tsunakawa, Yuki, Mizuno, Seiya, Yagami, Kenichi, Ishikawa, Chihiro, Li, Haiyan, Shiga, Takashi, Ishida, Junji, Hamada, Juri, Murata, Kazuya, Ishimaru, Tomohiro, Hashimoto, Misuzu, Fukamizu, Akiyoshi, Yamane, Mutsumi, Ikawa, Masahito, Morita, Hironobu, Shinohara, Masahiro, Asahara, Hiroshi, Akiyama, Taishin, Akiyama, Nobuko, Sasanuma, Hiroki, Yoshida, Nobuaki, Zhou, Rui, Wang, Ying-Ying, Ito, Taito, Kokubu, Yuko, Noguchi, Takaaki, K., Ishimine, Hisako, Kurisaki, Akira, Shiba, Dai, Mizuno, Hiroyasu, Shirakawa, Masaki, Ito, Naoki, Takeda, Shin, Takahashi, Satoru, 新保, 未来, 工藤, 崇, 濱田, 理人, 今村, 優希, 浅野, 圭吾, 岡田, 理沙, 綱川, 祐貴, 水野, 聖哉, 八神, 健一, 石川, 千尋, 志賀, 隆, 石田, 純治, 濱田, 樹理, 村田, 知弥, 石丸, 友博, 橋本, 美涼, 深水, 昭吉, 山根, 睦, 伊川, 正人, 森田, 啓之, 篠原, 正浩, 浅原, 弘嗣, 秋山, 泰身, 秋山, 伸子, 笹沼, 寛樹, 吉田, 進昭, 伊藤, 泰斗, 國分, 優子, 野口, 隆明, 石嶺, 久子, 栗崎, 晃, 芝, 大, 水野, 浩靖, 白川, 正輝, 伊藤, 尚基, 武田, 伸一, and 高橋, 智

Abstract

The Japan Aerospace Exploration Agency developed the mouse Habitat Cage Unit (HCU) for installation in the Cell Biology Experiment Facility (CBEF) onboard the Japanese Experimental Module (“Kibo”) on the International Space Station. The CBEF provides “space-based controls” by generating artificial gravity in the HCU through a centrifuge, enabling a comparison of the biological consequences of microgravity and artificial gravity of 1 g on mice housed in space. Therefore, prior to the space experiment, a ground-based study to validate the habitability of the HCU is necessary to conduct space experiments using the HCU in the CBEF. Here, we investigated the ground-based effect of a 32-day housing period in the HCU breadboard model on male mice in comparison with the control cage mice. Morphology of skeletal muscle, the thymus, heart, and kidney, and the sperm function showed no critical abnormalities between the control mice and HCU mice. Slight but significant changes caused by the HCU itself were observed, including decreased body weight, increased weights of the thymus and gastrocnemius, reduced thickness of cortical bone of the femur, and several gene expressions from 11 tissues. Results suggest that the HCU provides acceptable conditions for mouse phenotypic analysis using CBEF in space, as long as its characteristic features are considered. Thus, the HCU is a feasible device for future space experiments., full name: Takeda, Shinichi, Physical characteristics: Original contains illustrations, full name: 武田, 伸一, 形態: 図版あり

Published
2016

14. Ground-based assessment of JAXA mouse habitat cage unit by mouse phenotypic studies

Author

Shimbo, Miki, primary, Kudo, Takashi, additional, Hamada, Michito, additional, Jeon, Hyojung, additional, Imamura, Yuki, additional, Asano, Keigo, additional, Okada, Risa, additional, Tsunakawa, Yuki, additional, Mizuno, Seiya, additional, Yagami, Ken-ichi, additional, Ishikawa, Chihiro, additional, Li, Haiyan, additional, Shiga, Takashi, additional, Ishida, Junji, additional, Hamada, Juri, additional, Murata, Kazuya, additional, Ishimaru, Tomohiro, additional, Hashimoto, Misuzu, additional, Fukamizu, Akiyoshi, additional, Yamane, Mutsumi, additional, Ikawa, Masahito, additional, Morita, Hironobu, additional, Shinohara, Masahiro, additional, Asahara, Hiroshi, additional, Akiyama, Taishin, additional, Akiyama, Nobuko, additional, Sasanuma, Hiroki, additional, Yoshida, Nobuaki, additional, Zhou, Rui, additional, Wang, Ying-Ying, additional, Ito, Taito, additional, Kokubu, Yuko, additional, Noguchi, Taka-aki K., additional, Ishimine, Hisako, additional, Kurisaki, Akira, additional, Shiba, Dai, additional, Mizuno, Hiroyasu, additional, Shirakawa, Masaki, additional, Ito, Naoki, additional, Takeda, Shin, additional, and Takahashi, Satoru, additional

Published
2016
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15. Hypergravity Provokes a Temporary Reduction in CD4+CD8+ Thymocyte Number and a Persistent Decrease in Medullary Thymic Epithelial Cell Frequency in Mice

Author

Tateishi, Ryosuke, primary, Akiyama, Nobuko, additional, Miyauchi, Maki, additional, Yoshinaga, Riko, additional, Sasanuma, Hiroki, additional, Kudo, Takashi, additional, Shimbo, Miki, additional, Shinohara, Masahiro, additional, Obata, Koji, additional, Inoue, Jun-ichiro, additional, Shirakawa, Masaki, additional, Shiba, Dai, additional, Asahara, Hiroshi, additional, Yoshida, Nobuaki, additional, Takahashi, Satoru, additional, Morita, Hironobu, additional, and Akiyama, Taishin, additional

Published
2015
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18. Real-world safety and effectiveness of canakinumab in patients with tumour necrosis factor receptor-associated periodic syndrome or hyperimmunoglobulinaemia D syndrome: Interim results from post-marketing surveillance in Japan.

Author

Hosono K, Matsumoto K, Shimbo M, Tsumiyama I, and Kato C

Subjects
Humans, Japan, Syndrome, Product Surveillance, Postmarketing, Mevalonate Kinase Deficiency drug therapy, Familial Mediterranean Fever complications
Abstract

Objectives: To assess the real-world safety and effectiveness of canakinumab in patients in Japan with tumour necrosis factor receptor-associated periodic syndrome (TRAPS) or mevalonate kinase deficiency/hyperimmunoglobulinaemia D with periodic fever syndrome (MKD/HIDS)., Methods: All patients with TRAPS or MKD/HIDS who received canakinumab following drug approval in Japan were registered in a post-marketing all-patient surveillance with a 2-year observation period. Herein, the interim results are reported., Results: Fifteen patients with TRAPS and seven with MKD/HIDS were included in the safety and effectiveness analysis set. Adverse drug reactions were reported in 26.67% (n = 4) and 42.86% (n = 3) of TRAPS and MKD/HIDS patients, respectively. Most common adverse drug reactions were upper respiratory tract inflammation (13.33%, n = 2) and pyrexia (42.86%, n = 3) in TRAPS and MKD/HIDS patients, respectively. No serious adverse drug reactions were observed in either TRAPS or MKD/HIDS patients. The proportion of responders was 46.67% and 14.29% in the TRAPS and MKD/HIDS groups, respectively; 72.73% and 66.67% achieved clinical remission, while 90.91% and 66.67% achieved serological remission by Week 4 in the TRAPS and MKD/HIDS groups, respectively., Conclusions: These interim results provide the first evidence of the real-world effectiveness of canakinumab in patients with TRAPS or MKD/HIDS in Japan. No new safety concerns were identified., (© Japan College of Rheumatology 2022. Published by Oxford University Press.)

Published
2023
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