Fujita, Hibiki Kawamata

Publications

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selected publications

Accelerated transsulfuration metabolically defines a discrete subclass of amyotrophic lateral sclerosis patients. Neurobiology of disease. 2020 Academic Article GET IT
Times cited: 9
Glial mitochondrial function and dysfunction in health and neurodegeneration. Molecular and cellular neurosciences. 2019 Review GET IT
Times cited: 30
Nrf2 signaling links ER oxidative protein folding and calcium homeostasis in health and disease. Life science alliance. 2019 Academic Article GET IT
Times cited: 19
Parkin is a disease modifier in the mutant SOD1 mouse model of ALS. EMBO molecular medicine. 2018 Academic Article GET IT
Times cited: 43
In vitro and in vivo studies of the ALS-FTLD protein CHCHD10 reveal novel mitochondrial topology and protein interactions. Human molecular genetics. 2018 Academic Article GET IT
Times cited: 50
Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases. The Journal of cell biology. 2017 Review GET IT
Times cited: 52
Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo. Molecular neurodegeneration. 2017 Academic Article GET IT
Times cited: 29
Alterations in voltage-sensing of the mitochondrial permeability transition pore in ANT1-deficient cells. Scientific reports. 2016 Academic Article GET IT
Times cited: 32
Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis. Neurobiology of disease. 2015 Review GET IT
Times cited: 57
Bioenergetic markers in skin fibroblasts of sporadic amyotrophic lateral sclerosis and progressive lateral sclerosis patients. Annals of neurology. 2014 Academic Article GET IT
Times cited: 27
Abnormal intracellular calcium signaling and SNARE-dependent exocytosis contributes to SOD1G93A astrocyte-mediated toxicity in amyotrophic lateral sclerosis. The Journal of neuroscience : the official journal of the Society for Neuroscience. 2014 Academic Article GET IT
Times cited: 62
Measurement of ADP-ATP exchange in relation to mitochondrial transmembrane potential and oxygen consumption. Methods in enzymology. 2014 Academic Article GET IT
Times cited: 22
The negative impact of α-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation. FASEB journal : official publication of the Federation of American Societies for Experimental Biology. 2013 Academic Article GET IT
Times cited: 43
adPEO mutations in ANT1 impair ADP-ATP translocation in muscle mitochondria. Human molecular genetics. 2011 Academic Article GET IT
Times cited: 29
Introduction to neurodegenerative diseases and related techniques. Methods in molecular biology (Clifton, N.J.). 2011 Article GET IT
Times cited: 4
Import, maturation, and function of SOD1 and its copper chaperone CCS in the mitochondrial intermembrane space. Antioxidants & redox signaling. 2010 Review GET IT
Times cited: 105
A kinetic assay of mitochondrial ADP-ATP exchange rate in permeabilized cells. Analytical biochemistry. 2010 Academic Article GET IT
Times cited: 26
Mitochondrial dysfunction and intracellular calcium dysregulation in ALS. Mechanisms of ageing and development. 2010 Review GET IT
Times cited: 128
Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities. Human molecular genetics. 2009 Academic Article GET IT
Times cited: 125
Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria. The Journal of biological chemistry. 2008 Academic Article GET IT
Times cited: 37
Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria. Human molecular genetics. 2008 Academic Article GET IT
Times cited: 114
Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology. Proceedings of the National Academy of Sciences of the United States of America. 2007 Academic Article GET IT
Times cited: 126
Neurotoxicity and behavioral deficits associated with Septin 5 accumulation in dopaminergic neurons. Journal of neurochemistry. 2005 Academic Article GET IT
Times cited: 58
Experimental strategy to identify genes susceptible to oxidative stress in nigral dopaminergic neurons. Neurochemical research. 2004 Academic Article GET IT
Times cited: 12
Neural subtype specification of fertilization and nuclear transfer embryonic stem cells and application in parkinsonian mice. Nature biotechnology. 2003 Academic Article GET IT
Times cited: 538
TorsinA and heat shock proteins act as molecular chaperones: suppression of alpha-synuclein aggregation. Journal of neurochemistry. 2002 Academic Article GET IT
Times cited: 280
Alpha-synuclein has an altered conformation and shows a tight intermolecular interaction with ubiquitin in Lewy bodies. Acta neuropathologica. 2001 Academic Article GET IT
Times cited: 22
Interaction of alpha-synuclein and synphilin-1: effect of Parkinson's disease-associated mutations. Journal of neurochemistry. 2001 Academic Article GET IT
Times cited: 60
Alpha-synuclein-enhanced green fluorescent protein fusion proteins form proteasome sensitive inclusions in primary neurons. Neuroscience. 2001 Academic Article GET IT
Times cited: 190
Membrane association and protein conformation of alpha-synuclein in intact neurons. Effect of Parkinson's disease-linked mutations. The Journal of biological chemistry. 2000 Academic Article GET IT
Times cited: 217

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Research

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Funding awarded

Genetic and metabolic strategies for mutant CHCHD10 neuromuscular disorders awarded by Muscular Dystrophy Associations of America, Inc. Principal Investigator 2022 - 2025
Sponsored Research Agreement awarded by Relmada Therapeutics Principal Investigator 2022 - 2023
Pathogenic mechanisms and therapeutic strategies for CHCHD10 ALS awarded by Project ALS, Inc. Principal Investigator 2021 - 2023

Mitochondrial Integrated Stress Response In Neurological Diseases awarded by National Institute of Neurological Disorders & Stroke Co-Investigator 2021 - 2029
Amylyx SRA awarded by Amylyx Pharmaceuticals Co-Investigator 2023 - 2024
Amylyx: amx0035 (PBA + TUDCA) mechanisms of action awarded by Amylyx Pharmaceuticals Co-Investigator 2020 - 2023

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Background

education and training

Ph.D., Weill Cornell Graduate School of Medical Sciences 2008
B.A., Smith College 1999

Contact

full name

Hibiki Kawamata Fujita

primary email

Hik2004@med.cornell.edu

VIVO Weill Cornell Medical College

Hibiki Kawamata Fujita Assistant Professor of Research in Neuroscience

Publications

selected publications

Research

Funding awarded

Background

education and training

Contact

full name

primary email

Phone

Publications

selected publications

Research

Funding awarded

Background

education and training

Contact

full name

primary email