Publications

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.

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.

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

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.

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.

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.

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.

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

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.

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.

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.

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.

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.

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.

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.

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.

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

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)

Anderson CJ, Kahl A, Fruitman H, Qian L, Zhou P, Manfredi G, Iadecola C.  2019.  Prohibitin levels regulate OMA1 activity and turnover in neurons.. Cell Death Differ.

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

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.

Qu Y, Konrad C, Anderson C, Qian L, Yin T, Manfredi G, Iadecola C, Zhou P.  2020.  Prohibitin S-Nitrosylation Is Required for the Neuroprotective Effect of Nitric Oxide in Neuronal Cultures.. J Neurosci. 40(16):3142-3151.

Jenkins ECharles, Shah N, Gomez M, Casalena G, Zhao D, Kenny TC, Guariglia SRose, Manfredi G, Germain D.  2020.  Proteasome mapping reveals sexual dimorphism in tissue-specific sensitivity to protein aggregations.. EMBO Rep. :e48978.

Jenkins ECharles, Casalena G, Gomez M, Zhao D, Kenny TC, Shah N, Manfredi G, Germain D.  2020.  Raloxifene is a female-specific proteostasis therapeutic in the spinal cord.. Endocrinology.