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Found 16 results
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Kawamata, Hibiki
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A
Anderson CJ
,
Bredvik K
,
Burstein SR
,
Davis C
,
Meadows SM
,
Dash J
,
Case L
,
Milner TA
,
Kawamata H
,
Zuberi A
et al.
. 2019.
ALS/FTD mutant CHCHD10 mice reveal a tissue-specific toxic gain-of-function and mitochondrial stress response.
.
Acta Neuropathol.
Doczi J
,
Torocsik B
,
Echaniz-Laguna A
,
de Camaret BMousson
,
Starkov A
,
Starkova N
,
Gál A
,
Molnár MJ
,
Kawamata H
,
Manfredi G
et al.
. 2016.
Alterations in voltage-sensing of the mitochondrial permeability transition pore in ANT1-deficient cells.
.
Sci Rep. 6:26700.
D
Hayes LR
,
Asress SA
,
Li Y
,
Galkin A
,
Stepanova A
,
Kawamata H
,
Manfredi G
,
Glass JD
. 2019.
Distal denervation in the SOD1 knockout mouse correlates with loss of mitochondria at the motor nerve terminal.
.
Exp Neurol. 318:251-257.
E
Fels JA
,
Dash J
,
Leslie K
,
Manfredi G
,
Kawamata H
. 2022.
Effects of PB-TURSO on the transcriptional and metabolic landscape of sporadic ALS fibroblasts.
.
Ann Clin Transl Neurol.
F
Konrad C
,
Kawamata H
,
Bredvik KG
,
Arreguin AJ
,
Cajamarca SA
,
Hupf JC
,
Ravits JM
,
Miller TM
,
Maragakis NJ
,
Hales CM
et al.
. 2017.
Fibroblast bioenergetics to classify amyotrophic lateral sclerosis patients.
.
Mol Neurodegener. 12(1):76.
G
McAvoy K
,
Kawamata H
. 2019.
Glial mitochondrial function and dysfunction in health and neurodegeneration.
.
Mol Cell Neurosci. :103417.
Nelson AT
,
Cicardi MElena
,
Markandaiah SS
,
Han JYs
,
Philp NJ
,
Welebob E
,
Haeusler AR
,
Pasinelli P
,
Manfredi G
,
Kawamata H
et al.
. 2024.
Glucose hypometabolism prompts RAN translation and exacerbates C9orf72-related ALS/FTD phenotypes.
.
EMBO Rep. 25(5):2479-2510.
H
Southwell N
,
Manzo O
,
Bacman S
,
Zhao D
,
Sayles NM
,
Dash J
,
Fujita K
,
D'Aurelio M
,
Di Lorenzo A
,
Manfredi G
et al.
. 2024.
High fat diet ameliorates mitochondrial cardiomyopathy in CHCHD10 mutant mice.
.
EMBO Mol Med.
Southwell N
,
Zhao D
,
Sayles NM
,
Dash J
,
Fujita K
,
Aurelio MDâ
,
Manfredi G
,
Kawamata H
. 2023.
High fat diet ameliorates the mitochondrial cardiomyopathy of CHCHD10 mutant mice.
.
bioRxiv.
M
Manfredi G
,
Kawamata H
. 2016.
Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis.
.
Neurobiol Dis. 90:35-42.
Sayles NM
,
Southwell N
,
McAvoy K
,
Kim K
,
Pesini A
,
Anderson CJ
,
Quinzii C
,
Cloonan S
,
Kawamata H
,
Manfredi G
. 2022.
Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy.
.
Cell Rep. 38(10):110475.
Kawamata H
,
Peixoto P
,
Konrad C
,
Palomo G
,
Bredvik K
,
Gerges M
,
Valsecchi F
,
Petrucelli L
,
Ravits JM
,
Starkov A
et al.
. 2017.
Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo.
.
Mol Neurodegener. 12(1):37.
N
Granatiero V
,
Konrad C
,
Bredvik K
,
Manfredi G
,
Kawamata H
. 2019.
Nrf2 signaling links ER oxidative protein folding and calcium homeostasis in health and disease.
.
Life Sci Alliance. 2(5)
P
Palomo GM
,
Granatiero V
,
Kawamata H
,
Konrad C
,
Kim M
,
Arreguin AJ
,
Zhao D
,
Milner TA
,
Manfredi G
. 2018.
Parkin is a disease modifier in the mutant SOD1 mouse model of ALS.
.
EMBO Mol Med.
Kawamata H
,
Manfredi G
. 2017.
Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases.
.
J Cell Biol. 216(12):3917-3929.
S
Colognesi M
,
Shkodra A
,
Gabbia D
,
Kawamata H
,
Manfredi PL
,
Manfredi G
,
De Martin S
. 2024.
Sex-dependent effects of the uncompetitive N-methyl-D-aspartate receptor antagonist REL-1017 in G93A-SOD1 amyotrophic lateral sclerosis mice.
.
Front Neurol. 15:1384829.