Works matching AU Kawamata, Hibiki
Results: 21
ALS/FTD mutant CHCHD10 mice reveal a tissue-specific toxic gain-of-function and mitochondrial stress response.
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- Acta Neuropathologica, 2019, v. 138, n. 1, p. 103, doi. 10.1007/s00401-019-01989-y
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- Article
Glucose hypometabolism prompts RAN translation and exacerbates C9orf72-related ALS/FTD phenotypes.
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- EMBO Reports, 2024, v. 25, n. 5, p. 2479, doi. 10.1038/s44319-024-00140-7
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- Article
Proteinopathies and OXP HOS dysfunction in neurodegenerative diseases.
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- Journal of Cell Biology, 2017, v. 216, n. 12, p. 3917, doi. 10.1083/jcb.201709172
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- Article
Effects of PB‐TURSO on the transcriptional and metabolic landscape of sporadic ALS fibroblasts.
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- Annals of Clinical & Translational Neurology, 2022, v. 9, n. 10, p. 1551, doi. 10.1002/acn3.51648
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- Article
Abnormal Intracellular Calcium Signaling and SNAREDependent Exocytosis Contributes to SOD1G93A Astrocyte- Mediated Toxicity in Amyotrophic Lateral Sclerosis.
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- Journal of Neuroscience, 2014, v. 34, n. 6, p. 2331, doi. 10.1523/JNEUROSCI.2689-13.2014
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- Article
Fibroblast bioenergetics to classify amyotrophic lateral sclerosis patients.
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- Molecular Neurodegeneration, 2017, v. 12, p. 1, doi. 10.1186/s13024-017-0217-5
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- Article
Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo.
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- Molecular Neurodegeneration, 2017, v. 12, p. 1, doi. 10.1186/s13024-017-0180-1
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- Article
Bioenergetic markers in skin fibroblasts of sporadic amyotrophic lateral sclerosis and progressive lateral sclerosis patients.
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- Annals of Neurology, 2014, v. 76, n. 4, p. 620, doi. 10.1002/ana.24244
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- Article
Alpha-synuclein has an altered conformation and shows a tight intermolecular interaction with ubiquitin in Lewy bodies.
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- Acta Neuropathologica, 2001, v. 102, n. 4, p. 329, doi. 10.1007/s004010100369
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- Article
adPEO mutations in ANT1 impair ADP–ATP translocation in muscle mitochondria.
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- Human Molecular Genetics, 2011, v. 20, n. 15, p. 2964, doi. 10.1093/hmg/ddr200
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- Article
Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities.
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- Human Molecular Genetics, 2009, v. 18, n. 23, p. 4552, doi. 10.1093/hmg/ddp421
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- Article
Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria.
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- Human Molecular Genetics, 2008, v. 17, n. 21, p. 3303, doi. 10.1093/hmg/ddn226
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- Article
High fat diet ameliorates mitochondrial cardiomyopathy in CHCHD10 mutant mice.
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- EMBO Molecular Medicine, 2024, v. 16, n. 6, p. 1352, doi. 10.1038/s44321-024-00067-5
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- Article
Parkin is a disease modifier in the mutant SOD1 mouse model of ALS.
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- EMBO Molecular Medicine, 2018, v. 10, n. 10, p. N.PAG, doi. 10.15252/emmm.201808888
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- Article
Neural subtype specification of fertilization and nuclear transfer embryonic stem cells and application in parkinsonian mice.
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- Nature Biotechnology, 2003, v. 21, n. 10, p. 1200, doi. 10.1038/nbt870
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- Article
Modulation of the IGF1R-MTOR pathway attenuates motor neuron toxicity of human ALS SOD1<sup>G93A</sup> astrocytes.
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- Autophagy, 2021, v. 17, n. 12, p. 4029, doi. 10.1080/15548627.2021.1899682
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- Article
Neurotoxicity and behavioral deficits associated with.
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- Journal of Neurochemistry, 2005, v. 94, n. 4, p. 1040, doi. 10.1111/j.1471-4159.2005.03257.x
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- Article
Interaction of α-synuclein and synphilin-1: effect of Parkinson's disease-associated mutations.
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- Journal of Neurochemistry, 2001, v. 77, n. 3, p. 929, doi. 10.1046/j.1471-4159.2001.00301.x
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- Article
Sex-dependent effects of the uncompetitive N-methyl-D-aspartate receptor antagonist REL-1017 in G93A-SOD1 amyotrophic lateral sclerosis mice.
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- Frontiers in Neurology, 2024, p. 1, doi. 10.3389/fneur.2024.1384829
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- Article
Alterations in voltage-sensing of the mitochondrial permeability transition pore in ANT1-deficient cells.
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- Scientific Reports, 2016, p. 26700, doi. 10.1038/srep26700
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- Article
The negative impact of α-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation.
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- FASEB Journal, 2013, v. 27, n. 6, p. 2392, doi. 10.1096/fj.12-220202
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- Article