Search Results - "KAWAMATA, Hibiki"

Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis by Manfredi, Giovanni, Kawamata, Hibiki

“…Abstract Physical and functional interactions between mitochondria and the endoplasmic reticulum (ER) are crucial for cell life. These two organelles are…”
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Import, maturation, and function of SOD1 and its copper chaperone CCS in the mitochondrial intermembrane space by Kawamata, Hibiki, Manfredi, Giovanni

“…Cu, Zn, superoxide dismutase (SOD1) is a ubiquitous enzyme localized in multiple cellular compartments, including mitochondria, where it concentrates in the…”
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Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria by Kawamata, Hibiki, Manfredi, Giovanni

“…The antioxidant enzyme Cu,Zn superoxide dismutase (SOD1) is predominantly localized in the cytosol, but it is also found in mitochondria. Studies in yeast…”
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ALS/FTD mutant CHCHD10 mice reveal a tissue-specific toxic gain-of-function and mitochondrial stress response by Anderson, Corey J., Bredvik, Kirsten, Burstein, Suzanne R., Davis, Crystal, Meadows, Samantha M., Dash, Jalia, Case, Laure, Milner, Teresa A., Kawamata, Hibiki, Zuberi, Aamir, Piersigilli, Alessandra, Lutz, Cathleen, Manfredi, Giovanni

“…Mutations in coiled-coil-helix–coiled-coil-helix domain containing 10 (CHCHD10), a mitochondrial protein of unknown function, cause a disease spectrum with…”
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Parkin is a disease modifier in the mutant SOD1 mouse model of ALS by Palomo, Gloria M, Granatiero, Veronica, Kawamata, Hibiki, Konrad, Csaba, Kim, Michelle, Arreguin, Andrea J, Zhao, Dazhi, Milner, Teresa A, Manfredi, Giovanni

“…Mutant Cu/Zn superoxide dismutase (SOD1) causes mitochondrial alterations that contribute to motor neuron demise in amyotrophic lateral sclerosis (ALS). When…”
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Abnormal intracellular calcium signaling and SNARE-dependent exocytosis contributes to SOD1G93A astrocyte-mediated toxicity in amyotrophic lateral sclerosis by Kawamata, Hibiki, Ng, Seng Kah, Diaz, Natalia, Burstein, Suzanne, Morel, Lydie, Osgood, Alexandra, Sider, Brittany, Higashimori, Haruki, Haydon, Philip G, Manfredi, Giovanni, Yang, Yongjie

“…Motor neurons are progressively and predominantly degenerated in ALS, which is not only induced by multiple intrinsic pathways but also significantly…”
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Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy by Sayles, Nicole M., Southwell, Nneka, McAvoy, Kevin, Kim, Kihwan, Pesini, Alba, Anderson, Corey J., Quinzii, Catarina, Cloonan, Suzanne, Kawamata, Hibiki, Manfredi, Giovanni

“…Mitochondrial cardiomyopathies are fatal diseases, with no effective treatment. Alterations of heart mitochondrial function activate the mitochondrial…”
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High fat diet ameliorates mitochondrial cardiomyopathy in CHCHD10 mutant mice by Southwell, Nneka, Manzo, Onorina, Bacman, Sandra, Zhao, Dazhi, Sayles, Nicole M, Dash, Jalia, Fujita, Keigo, D’Aurelio, Marilena, Di Lorenzo, Annarita, Manfredi, Giovanni, Kawamata, Hibiki

“…Mutations in CHCHD10 , a mitochondrial protein with undefined functions, are associated with autosomal dominant mitochondrial diseases. Chchd10 knock-in mice…”
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Sex-dependent effects of the uncompetitive N-methyl-D-aspartate receptor antagonist REL-1017 in G93A-SOD1 amyotrophic lateral sclerosis mice by Colognesi, Martina, Shkodra, Atea, Gabbia, Daniela, Kawamata, Hibiki, Manfredi, Paolo L, Manfredi, Giovanni, De Martin, Sara

“…The pathogenesis of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease caused by the demise of motor neurons has been linked to…”
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Effects of PB‐TURSO on the transcriptional and metabolic landscape of sporadic ALS fibroblasts by Fels, Jasmine A., Dash, Jalia, Leslie, Kent, Manfredi, Giovanni, Kawamata, Hibiki

“…Objective ALS is a rapidly progressive, fatal disorder caused by motor neuron degeneration, for which there is a great unmet therapeutic need. AMX0035, a…”
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Accelerated transsulfuration metabolically defines a discrete subclass of amyotrophic lateral sclerosis patients by Chen, Qiuying, Konrad, Csaba, Sandhu, Davinder, Roychoudhury, Dipa, Schwartz, Benjamin I., Cheng, Roger R., Bredvik, Kirsten, Kawamata, Hibiki, Calder, Elizabeth L., Studer, Lorenz, Fischer, Steven.M., Manfredi, Giovanni, Gross, Steven.S.

“…Amyotrophic lateral sclerosis is a disease characterized by progressive paralysis and death. Most ALS-cases are sporadic (sALS) and patient heterogeneity poses…”
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Fibroblast bioenergetics to classify amyotrophic lateral sclerosis patients by Konrad, Csaba, Kawamata, Hibiki, Bredvik, Kirsten G, Arreguin, Andrea J, Cajamarca, Steven A, Hupf, Jonathan C, Ravits, John M, Miller, Timothy M, Maragakis, Nicholas J, Hales, Chadwick M, Glass, Jonathan D, Gross, Steven, Mitsumoto, Hiroshi, Manfredi, Giovanni

“…The objective of this study was to investigate cellular bioenergetics in primary skin fibroblasts derived from patients with amyotrophic lateral sclerosis…”
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Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology by Son, Marjatta, Puttaparthi, Krishna, Kawamata, Hibiki, Rajendran, Bhagya, Boyer, Philip J, Manfredi, Giovanni, Elliott, Jeffrey L

“…Cu, Zn superoxide dismutase (SOD1) has been detected within spinal cord mitochondria of mutant SOD1 transgenic mice, a model of familial ALS. The copper…”
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Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo by Kawamata, Hibiki, Peixoto, Pablo, Konrad, Csaba, Palomo, Gloria, Bredvik, Kirsten, Gerges, Meri, Valsecchi, Federica, Petrucelli, Leonard, Ravits, John M, Starkov, Anatoly, Manfredi, Giovanni

“…Mitochondrial dysfunction has been linked to the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Functional…”
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Neural subtype specification of fertilization and nuclear transfer embryonic stem cells and application in parkinsonian mice by Perrier, Anselme L, Topf, Norbert, Barberi, Tiziano, Rubio, Maria E, Calingasan, Noel Y, Bruses, Juan, Studer, Lorenz, Klivenyi, Peter, Beal, M Flint, Moore, Malcolm A S, Kawamata, Hibiki, Harrison, Neil L, Lee, Hyojin, Loonam, Kathleen, Tabar, Viviane

“…Existing protocols for the neural differentiation of mouse embryonic stem (ES) cells require extended in vitro culture, yield variable differentiation results…”
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A kinetic assay of mitochondrial ADP–ATP exchange rate in permeabilized cells by Kawamata, Hibiki, Starkov, Anatoly A., Manfredi, Giovanni, Chinopoulos, Christos

“…We previously described a method to measure ADP–ATP exchange rates in isolated mitochondria by recording the changes in free extramitochondrial [Mg 2+]…”
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Lysyl-tRNA Synthetase Is a Target for Mutant SOD1 Toxicity in Mitochondria by Kawamata, Hibiki, Magrané, Jordi, Kunst, Catherine, King, Michael P., Manfredi, Giovanni

“…Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease affecting the motor neurons. The majority of familial forms of ALS are caused by…”
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Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases by Kawamata, Hibiki, Manfredi, Giovanni

“…Mitochondria participate in essential processes in the nervous system such as energy and intermediate metabolism, calcium homeostasis, and apoptosis. Major…”
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Glial mitochondrial function and dysfunction in health and neurodegeneration by McAvoy, Kevin, Kawamata, Hibiki

“…Mitochondria play essential metabolic roles in neural cells. Mitochondrial dysfunction has profound effects on the brain. In primary mitochondrial diseases,…”
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Mitochondrial dysfunction and intracellular calcium dysregulation in ALS by Kawamata, Hibiki, Manfredi, Giovanni

“…Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder that affects the aging population. A progressive loss of motor neurons in the…”
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