Search Results - "H Maegawa"

Antigen-encapsulating host extracellular vesicles derived from Salmonella-infected cells stimulate pathogen-specific Th1-type responses in vivo by Hui, Winnie W, Emerson, Lisa E, Clapp, Beata, Sheppe, Austin E, Sharma, Jatin, Del Castillo, Johanna, Ou, Mark, Maegawa, Gustavo H B, Hoffman, Carol, Larkin Iii, Joseph, Pascual, David W, Ferraro, Mariola J

“…Salmonella Typhimurium is a causative agent of nontyphoidal salmonellosis, for which there is a lack of a clinically approved vaccine in humans. As an…”
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CNS-Targeting Therapies for Lysosomal Storage Diseases: Current Advances and Challenges by Edelmann, Mariola J., Maegawa, Gustavo H. B.

“…During the past decades, several therapeutic approaches have been developed and made rapidly available for many patients afflicted with lysosomal storage…”
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Identification and Characterization of Ambroxol as an Enzyme Enhancement Agent for Gaucher Disease by Maegawa, Gustavo H.B., Tropak, Michael B., Buttner, Justin D., Rigat, Brigitte A., Fuller, Maria, Pandit, Deepangi, Tang, Liangiie, Kornhaber, Gregory J., Hamuro, Yoshitomo, Clarke, Joe T.R., Mahuran, Don J.

“…Gaucher disease (GD), the most prevalent lysosomal storage disease, is caused by a deficiency of glucocerebrosidase (GCase). The identification of small…”
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Early axonal loss accompanied by impaired endocytosis, abnormal axonal transport, and decreased microtubule stability occur in the model of Krabbe's disease by Teixeira, Carla Andreia, Miranda, Catarina Oliveira, Sousa, Vera Filipe, Santos, Telma Emanuela, Malheiro, Ana Rita, Solomon, Melani, Maegawa, Gustavo H, Brites, Pedro, Sousa, Mónica Mendes

“…Abstract In Krabbe's disease (KD), a leukodystrophy caused by β-galactosylceramidase deficiency, demyelination and a myelin-independent axonopathy contributes…”
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Clinical, biochemical, and genotype‐phenotype correlations of 118 patients with Niemann‐Pick disease Types A/B by Hu, Jiayue, Maegawa, Gustavo H.B., Zhan, Xia, Gao, Xiaolan, Wang, Yu, Xu, Feng, Qiu, Wenjuan, Han, Lianshu, Gu, Xuefan, Zhang, Huiwen

“…Niemann‐Pick disease Types A and B (NPA/B) are autosomal recessive disorders caused by variants in the sphingomyelin phosphodiesterase‐1 (SMPD1) gene. This…”
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Ambroxol improves skeletal and hematological manifestations on a child with Gaucher disease by Jiang, Wenjun, Yi, Mengni, Maegawa, Gustavo H B, Zhang, Huiwen

“…Gaucher disease (GD) is a lysosomal storage disease caused by the deficiency of glucocerebrosidase characterized by a broad spectrum of clinical manifestations…”
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Chronic kidney disease categories and renal–cardiovascular outcomes in type 2 diabetes without prevalent cardiovascular disease: a prospective cohort study (JDDM25) by Yokoyama, H., Araki, S., Haneda, M., Matsushima, M., Kawai, K., Hirao, K., Oishi, M., Sugimoto, K., Sone, H., Maegawa, H., Kashiwagi, A.

“…Aims/hypothesis In type 2 diabetic patients at low risk for cardiovascular disease (CVD), the relationship between the clinical course of nephropathy by stage…”
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USP8 inhibition regulates autophagy flux and controls Salmonella infection by Santelices, John, Ou, Mark, Maegawa, Gustavo H B, Hercik, Kamil, Edelmann, Mariola J

“…Ubiquitination is an important protein modification that regulates various essential cellular processes, including the functions of innate immune cells…”
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A diagnosis of non‐neuronopathic and late‐onset acid sphingomyelinase deficiency (Niemann‐Pick disease A/B) following bone marrow biopsy showing foamy histiocytosis by SahBandar, Ivo N., Maegawa, Gustavo H. B., Brandman, Danielle, Rand, Jacob H., Lim, Hana I., Geyer, Julia T.

“…SEE PDF] The clinical history and bone marrow biopsy findings were suspicious for lysosomal storage disease, and subsequent sphingomyelinase enzymatic activity…”
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The Natural History of Juvenile or Subacute GM2 Gangliosidosis: 21 New Cases and Literature Review of 134 Previously Reported by Maegawa, Gustavo H. B, Stockley, Tracy, Tropak, Michael, Banwell, Brenda, Blaser, Susan, Kok, Fernando, Giugliani, Roberto, Mahuran, Don, Clarke, Joe T.R

“…Juvenile GM2 gangliosidosis is a group of inherited neurodegenerative diseases caused by deficiency of lysosomal beta-hexosaminidase resulting in GM2…”
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Japanese men have larger areas of visceral adipose tissue than Caucasian men in the same levels of waist circumference in a population-based study by Kadowaki, T, Sekikawa, A, Murata, K, Maegawa, H, Takamiya, T, Okamura, T, El-Saed, A, Miyamatsu, N, Edmundowicz, D, Kita, Y

“…Visceral adipose tissue (VAT) is an independent risk factor for metabolic and cardiovascular disorders. There has been no study that demonstrated different…”
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Use of Ambroxol as Therapy for Gaucher Disease by Zhan, Xia, Zhang, Huiwen, Maegawa, Gustavo H B, Wang, Yu, Gao, Xiaolan, Wang, Dengbin, Li, Jinning

“…Ambroxol was identified as an enhancer of stability and residual activity of several misfolded glucocerebrosidase variants in 2009. To assess hematologic and…”
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Pyrimethamine as a Potential Pharmacological Chaperone for Late-onset Forms of GM2 Gangliosidosis by Maegawa, Gustavo H.B., Tropak, Michael, Buttner, Justin, Stockley, Tracy, Kok, Fernando, Clarke, Joe T.R., Mahuran, Don J.

“…Late-onset GM2 gangliosidosis is composed of two related, autosomal recessive, neurodegenerative diseases, both resulting from deficiency of lysosomal,…”
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Safety and effectiveness of ipragliflozin in Japanese patients with type 2 diabetes mellitus and impaired renal function: subgroup analysis of a 3-year post-marketing surveillance study (STELLA-LONG TERM) by Tobe, K., Maegawa, H., Nakamura, I., Uno, S.

“…STELLA-LONG TERM, a 3-year post-marketing surveillance study, evaluated the safety and effectiveness of the sodiumglucose cotransporter 2 inhibitor…”
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Potential Disease-Modifying Effects of Lithium Carbonate in Niemann-Pick Disease, Type C1 by Han, Shiqian, Zhang, Huiwen, Yi, Mengni, Liu, Xiaoqing, Maegawa, Gustavo H. B., Zou, Yunding, Wang, Qijun, Wu, Dianqing, Ye, Zhijia

“…Background: Niemann-Pick disease type C1 (NP-C1) is a rare, autosomal-recessive neurodegenerative disorder with no United States Food and Drug Administration…”
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Cardiovascular disease management in people with diabetes outside North America and Western Europe in 2006 and 2015 by Tabesh, M., Magliano, D. J., Tanamas, S. K., Surmont, F., Bahendeka, S., Chiang, C.‐E., Elgart, J. F., Gagliardino, J. J., Kalra, S., Krishnamoorthy, S., Luk, A., Maegawa, H., Motala, A. A., Pirie, F., Ramachandran, A., Tayeb, K., Vikulova, O., Wong, J., Shaw, J. E.

“…Aim Optimal treatment of cardiovascular disease is essential to decrease mortality among people with diabetes, but information is limited on how actual…”
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Isofagomine Induced Stabilization of Glucocerebrosidase by Kornhaber, Gregory J, Tropak, Michael B, Maegawa, Gustavo H, Tuske, Steven J, Coales, Stephen J, Mahuran, Don J, Hamuro, Yoshitomo

“…Structurally destabilizing mutations in acid β-glucosidase (GCase) can result in Gaucher disease (GD). The iminosugar isofagomine (IFG), a competitive…”
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Pioglitazone treatment and cardiovascular event and death in subjects with type 2 diabetes without established cardiovascular disease (JDDM 36) by Yokoyama, H, Araki, S, Kawai, K, Hirao, K, Oishi, M, Sugimoto, K, Sone, H, Maegawa, H, Kashiwagi, A

“…Abstract Aims The protective association of pioglitazone with cardiovascular events and death was investigated over 6-years in large-scale type 2 diabetic…”
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Less Subclinical Atherosclerosis in Japanese Men in Japan than in White Men in the United States in the Post–World War II Birth Cohort by Sekikawa, Akira, Ueshima, Hirotsugu, Kadowaki, Takashi, El-Saed, Aiman, Okamura, Tomonori, Takamiya, Tomoko, Kashiwagi, Atsunori, Edmundowicz, Daniel, Murata, Kiyoshi, Sutton-Tyrrell, Kim, Maegawa, Hiroshi, Evans, Rhobert W., Kita, Yoshikuni, Kuller, Lewis H.

“…Coronary heart disease incidence and mortality remain very low in Japan despite major dietary changes and increases in risk factors that should have resulted…”
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Genetic variants at CDC123/CAMK1D and SPRY2 are associated with susceptibility to type 2 diabetes in the Japanese population by Imamura, M., Iwata, M., Maegawa, H., Watada, H., Hirose, H., Tanaka, Y., Tobe, K., Kaku, K., Kashiwagi, A., Kawamori, R., Nakamura, Y., Maeda, S.

“…Aims/hypothesis Recently, rs10906115 in CDC123/CAMK1D , rs1359790 near SPRY2 , rs1436955 in C2CD4A/C2CD4B and rs10751301 in ODZ4 were identified as genetic…”
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