Publications

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)

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.

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.

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)

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

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.

Sasset L, Chowdhury KH, Manzo OL, Rubinelli L, Konrad C, J Maschek A, Manfredi G, Holland WL, Di Lorenzo A.  2023.  Sphingosine-1-phosphate controls endothelial sphingolipid homeostasis via ORMDL.. EMBO Rep. 24(1):e54689.

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.

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.

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.

Tracy TE, Madero-Pérez J, Swaney DL, Chang TS, Moritz M, Konrad C, Ward ME, Stevenson E, Hüttenhain R, Kauwe G et al..  2022.  Tau interactome maps synaptic and mitochondrial processes associated with neurodegeneration.. Cell. 185(4):712-728.e14.

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, Casalena GA, Manfredi G.  2021.  Sex and oestrogen receptor β have modest effects on gene expression in the mouse brain posterior cortex.. Endocrinol Diabetes Metab. 4(1):e00191.

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.

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)