Publications

Fels JA, Manfredi G.  2019.  Sex Differences in Ischemia/Reperfusion Injury: The Role of Mitochondrial Permeability Transition.. Neurochem Res.

Chen Q, Kirk K, Shurubor YI, Zhao D, Arreguin AJ, Shahi I, Valsecchi F, Primiano G, Calder EL, Carelli V et al..  2018.  Rewiring of Glutamine Metabolism Is a Bioenergetic Adaptation of Human Cells with Mitochondrial DNA Mutations.. Cell Metab.

Kawamata H, Manfredi G.  2017.  Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases.. J Cell Biol. 216(12):3917-3929.

Anderson CJ, Kahl A, Qian L, Stepanova A, Starkov A, Manfredi G, Iadecola C, Zhou P.  2018.  Prohibitin is a positive modulator of mitochondrial function in PC12 cells under oxidative stress.. J Neurochem.

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.

Kuszak AJ, Espey MGraham, Falk MJ, Holmbeck MA, Manfredi G, Shadel GS, Vernon HJ, Zolkipli-Cunningham Z.  2017.  Nutritional Interventions for Mitochondrial OXPHOS Deficiencies: Mechanisms and Model Systems.. Annu Rev Pathol.

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)

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.

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.

Granatiero V, Manfredi G.  2019.  Mitochondrial Transport and Turnover in the Pathogenesis of Amyotrophic Lateral Sclerosis.. Biology (Basel). 8(2)

Manfredi G, Kawamata H.  2016.  Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis.. Neurobiol Dis. 90:35-42.

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.

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.

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.

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.

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.

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.

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.

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.

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.