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Found 40 results
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Manfredi, Giovanni
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Journal Article
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.
Deng Z
,
Lim J
,
Wang Q
,
Purtell K
,
Wu S
,
Palomo GM
,
Tan H
,
Manfredi G
,
Zhao Y
,
Peng J
et al.
. 2019.
ALS-FTLD-linked mutations of SQSTM1/p62 disrupt selective autophagy and NFE2L2/NRF2 anti-oxidative stress pathway.
.
Autophagy. :1-15.
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.
P Ozdinler H
,
Gautam M
,
Gozutok O
,
Konrad C
,
Manfredi G
,
Gomez EArea
,
Mitsumoto H
,
Erb ML
,
Tian Z
,
Haase G
. 2020.
Better understanding the neurobiology of primary lateral sclerosis.
.
Amyotroph Lateral Scler Frontotemporal Degener. 21(sup1):35-46.
Sayles NM
,
Napierala JS
,
Anrather J
,
Diedhiou N
,
Li J
,
Napierala M
,
Puccio H
,
Manfredi G
. 2023.
Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency.
.
Dis Model Mech. 16(10)
Sayles NM
,
Napierala JS
,
Anrather J
,
Diedhiou N
,
Li J
,
Napierala M
,
Puccio H
,
Manfredi G
. 2024.
Correction: Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency.
.
Dis Model Mech. 17(1)
Stepanova A
,
Konrad C
,
Manfredi G
,
Springett R
,
Ten V
,
Galkin A
. 2018.
The dependence of brain mitochondria reactive oxygen species production on oxygen level is linear, except when inhibited by antimycin A.
.
J Neurochem.
Stepanova A
,
Shurubor Y
,
Valsecchi F
,
Manfredi G
,
Galkin A
. 2016.
Differential susceptibility of mitochondrial complex II to inhibition by oxaloacetate in brain and heart.
.
Biochim Biophys Acta. 1857(9):1561-8.
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.
Jenkins ECharles
,
O'Connell MJ
,
Manfredi G
,
Germain D
. 2021.
Doxycycline promotes proteasome fitness in the central nervous system.
.
Sci Rep. 11(1):17003.
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.
Burstein SR
,
Kim HJeong
,
Fels JA
,
Qian L
,
Zhang S
,
Zhou P
,
Starkov AA
,
Iadecola C
,
Manfredi G
. 2018.
Estrogen receptor beta modulates permeability transition in brain mitochondria.
.
Biochim Biophys Acta.
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.
Fels JA
,
Casalena G
,
Konrad C
,
Holmes HE
,
Dellinger RW
,
Manfredi G
. 2022.
Gene expression profiles in sporadic ALS fibroblasts define disease subtypes and the metabolic effects of the investigational drug EH301.
.
Hum Mol Genet.
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.
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.
Yang S
,
Le Kernec J
,
Romain O
,
Fioranelli F
,
Cadart P
,
Fix J
,
Ren C
,
Manfredi G
,
Letertre T
,
Saenz IDavid Hino
et al.
. 2023.
The Human Activity Radar Challenge: benchmarking based on the 'Radar signatures of human activities' dataset from Glasgow University.
.
IEEE J Biomed Health Inform. PP
Cornacchia D
,
Zhang C
,
Zimmer B
,
Chung SYoung
,
Fan Y
,
Soliman MA
,
Tchieu J
,
Chambers SM
,
Shah H
,
Paull D
et al.
. 2019.
Lipid Deprivation Induces a Stable, Naive-to-Primed Intermediate State of Pluripotency in Human PSCs.
.
Cell Stem Cell.
Woo E
,
Bredvik K
,
Liu B
,
Fuchs TJ
,
Manfredi G
,
Konrad C
. 2023.
Machine learning approaches based on fibroblast morphometry do not predict ALS.
.
Neurobiol Aging. 130:80-83.
Manfredi G
,
Kawamata H
. 2016.
Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis.
.
Neurobiol Dis. 90:35-42.
Granatiero V
,
Manfredi G
. 2019.
Mitochondrial Transport and Turnover in the Pathogenesis of Amyotrophic Lateral Sclerosis.
.
Biology (Basel). 8(2)
Kenny TC
,
Manfredi G
,
Germain D
. 2017.
The Mitochondrial Unfolded Protein Response as a Non-Oncogene Addiction to Support Adaptation to Stress during Transformation in Cancer and Beyond.
.
Front Oncol. 7:159.
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.
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