Feil Family Brain & Mind Research Institute

Publications

Found 16 results

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Filters: Author is Kawamata, Hibiki [Clear All Filters]

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.

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.

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.

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.

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.

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.

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.

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.

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)

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.

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.