| Year |
Citation |
Score |
| 2024 |
Liao SC, Kano K, Phanse S, Nguyen M, Margolis E, Fu Y, Meng J, Moutaoufik MT, Chatterton Z, Aoki H, Simms J, Hsieh I, Suteja F, Sei Y, Huang EJ, ... ... Manfredi G, et al. CHCHD2 mutant mice display mitochondrial protein accumulation and disrupted energy metabolism. Biorxiv : the Preprint Server For Biology. PMID 39257750 DOI: 10.1101/2024.08.30.610586 |
0.44 |
|
| 2024 |
Southwell N, Manzo O, Bacman S, Zhao D, Sayles NM, Dash J, Fujita K, D'Aurelio M, Di Lorenzo A, Manfredi G, Kawamata H. High fat diet ameliorates mitochondrial cardiomyopathy in CHCHD10 mutant mice. Embo Molecular Medicine. PMID 38724625 DOI: 10.1038/s44321-024-00067-5 |
0.431 |
|
| 2024 |
Sayles NM, Napierala JS, Anrather J, Diedhiou N, Li J, Napierala M, Puccio H, Manfredi G. Correction: Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency. Disease Models & Mechanisms. 17. PMID 38214340 DOI: 10.1242/dmm.050689 |
0.333 |
|
| 2023 |
Southwell N, Primiano G, Nadkarni V, Attarwala N, Beattie E, Miller D, Alam S, Liparulo I, Shurubor YI, Valentino ML, Carelli V, Servidei S, Gross SS, Manfredi G, Chen Q, et al. A coordinated multiorgan metabolic response contributes to human mitochondrial myopathy. Embo Molecular Medicine. e16951. PMID 37222423 DOI: 10.15252/emmm.202216951 |
0.409 |
|
| 2023 |
Southwell N, Zhao D, Sayles NM, Dash J, Fujita K, Dâ Aurelio M, Manfredi G, Kawamata H. High fat diet ameliorates the mitochondrial cardiomyopathy of CHCHD10 mutant mice. Biorxiv : the Preprint Server For Biology. PMID 36865125 DOI: 10.1101/2023.02.22.529577 |
0.419 |
|
| 2022 |
Sayles NM, Southwell N, McAvoy K, Kim K, Pesini A, Anderson CJ, Quinzii C, Cloonan S, Kawamata H, Manfredi G. Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy. Cell Reports. 38: 110475. PMID 35263592 DOI: 10.1016/j.celrep.2022.110475 |
0.459 |
|
| 2021 |
Jenkins EC, O'Connell MJ, Manfredi G, Germain D. Doxycycline promotes proteasome fitness in the central nervous system. Scientific Reports. 11: 17003. PMID 34417525 DOI: 10.1038/s41598-021-96540-z |
0.392 |
|
| 2020 |
Qu Y, Conrad C, Anderson C, Qian L, Yin T, Manfredi G, Iadecola C, Zhou P. Prohibitin S-nitrosylation is required for the neuroprotective effect of nitric oxide in neuronal cultures. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. PMID 32152200 DOI: 10.1523/Jneurosci.1804-19.2020 |
0.434 |
|
| 2019 |
Anderson CJ, Kahl A, Fruitman H, Qian L, Zhou P, Manfredi G, Iadecola C. Prohibitin levels regulate OMA1 activity and turnover in neurons. Cell Death and Differentiation. PMID 31819158 DOI: 10.1038/S41418-019-0469-4 |
0.515 |
|
| 2019 |
Deng Z, Lim J, Wang Q, Purtell K, Wu S, Palomo GM, Tan H, Manfredi G, Zhao Y, Peng J, Hu B, Chen S, Yue Z. ALS-FTLD-linked mutations of SQSTM1/p62 disrupt selective autophagy and NFE2L2/NRF2 anti-oxidative stress pathway. Autophagy. 1-15. PMID 31362587 DOI: 10.1080/15548627.2019.1644076 |
0.398 |
|
| 2019 |
Witherspoon M, Sandu D, Lu C, Wang K, Edwards R, Yeung A, Gelincik O, Manfredi G, Gross S, Kopelovich L, Lipkin S. ETHE1 overexpression promotes SIRT1 and PGC1α mediated aerobic glycolysis, oxidative phosphorylation, mitochondrial biogenesis and colorectal cancer. Oncotarget. 10: 4004-4017. PMID 31258845 DOI: 10.18632/Oncotarget.26958 |
0.391 |
|
| 2019 |
Granatiero V, Manfredi G. Mitochondrial Transport and Turnover in the Pathogenesis of Amyotrophic Lateral Sclerosis. Biology. 8. PMID 31083575 DOI: 10.3390/Biology8020036 |
0.52 |
|
| 2019 |
Hayes LR, Asress SA, Li Y, Galkin A, Stepanova A, Kawamata H, Manfredi G, Glass JD. Distal denervation in the SOD1 knockout mouse correlates with loss of mitochondria at the motor nerve terminal. Experimental Neurology. PMID 31082391 DOI: 10.1016/J.Expneurol.2019.05.008 |
0.501 |
|
| 2019 |
Stepanova A, Sosunov S, Niatsetskaya Z, Konrad C, Starkov AA, Manfredi G, Wittig I, Ten V, Galkin A. Redox-dependent loss of flavin by mitochondrial complex I in brain ischemia/reperfusion injury. Antioxidants & Redox Signaling. PMID 31037949 DOI: 10.1089/Ars.2018.7693 |
0.508 |
|
| 2019 |
Anderson CJ, Bredvik K, Burstein SR, Davis C, Meadows SM, Dash J, Case L, Milner TA, Kawamata H, Zuberi A, Piersigilli A, Lutz C, Manfredi G. ALS/FTD mutant CHCHD10 mice reveal a tissue-specific toxic gain-of-function and mitochondrial stress response. Acta Neuropathologica. PMID 30877432 DOI: 10.1007/S00401-019-01989-Y |
0.494 |
|
| 2019 |
Fels JA, Manfredi G. Sex Differences in Ischemia/Reperfusion Injury: The Role of Mitochondrial Permeability Transition. Neurochemical Research. PMID 30863968 DOI: 10.1007/S11064-019-02769-6 |
0.469 |
|
| 2018 |
Stepanova A, Konrad C, Manfredi G, Springett R, Ten V, Galkin A. The dependence of brain mitochondria reactive oxygen species production on oxygen level is linear, except when inhibited by antimycin A. Journal of Neurochemistry. PMID 30582748 DOI: 10.1111/Jnc.14654 |
0.412 |
|
| 2018 |
Song M, Sandoval TA, Chae CS, Chopra S, Tan C, Rutkowski MR, Raundhal M, Chaurio RA, Payne KK, Konrad C, Bettigole SE, Shin HR, Crowley MJP, Cerliani JP, Kossenkov AV, ... ... Manfredi G, et al. IRE1α-XBP1 controls T cell function in ovarian cancer by regulating mitochondrial activity. Nature. PMID 30305738 DOI: 10.1038/S41586-018-0597-X |
0.371 |
|
| 2018 |
Palomo GM, Granatiero V, Kawamata H, Konrad C, Kim M, Arreguin AJ, Zhao D, Milner TA, Manfredi G. Parkin is a disease modifier in the mutant SOD1 mouse model of ALS. Embo Molecular Medicine. PMID 30126943 DOI: 10.15252/Emmm.201808888 |
0.587 |
|
| 2018 |
Anderson CJ, Kahl A, Qian L, Stepanova A, Starkov A, Manfredi G, Iadecola C, Zhou P. Prohibitin is a positive modulator of mitochondrial function in PC12 cells under oxidative stress. Journal of Neurochemistry. PMID 29808474 DOI: 10.1111/Jnc.14472 |
0.563 |
|
| 2018 |
Chen Q, Kirk K, Shurubor YI, Zhao D, Arreguin AJ, Shahi I, Valsecchi F, Primiano G, Calder EL, Carelli V, Denton TT, Beal MF, Gross SS, Manfredi G, D'Aurelio M. Rewiring of Glutamine Metabolism Is a Bioenergetic Adaptation of Human Cells with Mitochondrial DNA Mutations. Cell Metabolism. PMID 29657030 DOI: 10.1016/J.Cmet.2018.03.002 |
0.568 |
|
| 2018 |
Kahl A, Stepanova A, Konrad C, Anderson C, Manfredi G, Zhou P, Iadecola C, Galkin A. Critical Role of Flavin and Glutathione in Complex I-Mediated Bioenergetic Failure in Brain Ischemia/Reperfusion Injury. Stroke. PMID 29643256 DOI: 10.1161/Strokeaha.117.019687 |
0.341 |
|
| 2018 |
Stepanova A, Konrad C, Guerrero-Castillo S, Manfredi G, Vannucci S, Arnold S, Galkin A. Deactivation of mitochondrial complex I after hypoxia-ischemia in the immature brain. Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism. 271678X18770331. PMID 29629602 DOI: 10.1177/0271678X18770331 |
0.516 |
|
| 2018 |
Burstein SR, Kim HJ, Fels JA, Qian L, Zhang S, Zhou P, Starkov AA, Iadecola C, Manfredi G. Estrogen receptor beta modulates permeability transition in brain mitochondria. Biochimica Et Biophysica Acta. PMID 29550215 DOI: 10.1016/J.Bbabio.2018.03.006 |
0.429 |
|
| 2018 |
Kawamata H, Manfredi G. Correction: Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases. The Journal of Cell Biology. 217: 429. PMID 29269424 DOI: 10.1083/Jcb.20170917212192017C |
0.4 |
|
| 2018 |
Stepanova A, Konrad C, Manfredi G, Ten V, Galkin A. Reactive oxygen species production by brain mitochondria depends linearly on oxygen level Free Radical Biology and Medicine. 120: S136. DOI: 10.1016/J.Freeradbiomed.2018.04.447 |
0.425 |
|
| 2018 |
Galkin A, Stepanova A, Konrad C, Manfredi G, Ten V. Mitochondrial Reactive Oxygen Species Production and Oxygen Level: Linear Dependence or Not? Biochimica Et Biophysica Acta (Bba) - Bioenergetics. 1859: e12. DOI: 10.1016/J.Bbabio.2018.09.038 |
0.392 |
|
| 2017 |
Connolly NMC, Theurey P, Adam-Vizi V, Bazan NG, Bernardi P, Bolaños JP, Culmsee C, Dawson VL, Deshmukh M, Duchen MR, Düssmann H, Fiskum G, Galindo MF, Hardingham GE, Hardwick JM, ... ... Manfredi G, et al. Guidelines on experimental methods to assess mitochondrial dysfunction in cellular models of neurodegenerative diseases. Cell Death and Differentiation. PMID 29229998 DOI: 10.1038/S41418-017-0020-4 |
0.524 |
|
| 2017 |
Kawamata H, Manfredi G. Proteinopathies and OXPHOS dysfunction in neurodegenerative diseases. The Journal of Cell Biology. 216: 3917-3929. PMID 29167179 DOI: 10.1083/Jcb.201709172 |
0.522 |
|
| 2017 |
Burstein SR, Valsecchi F, Kawamata H, Bourens M, Zeng R, Zuberi A, Milner TA, Cloonan SM, Lutz C, Barrientos A, Manfredi G. In vitro and in vivo studies of the ALS-FTLD protein CHCHD10 reveal novel mitochondrial topology and protein interactions. Human Molecular Genetics. PMID 29112723 DOI: 10.1093/Hmg/Ddx397 |
0.565 |
|
| 2017 |
Kuszak AJ, Espey MG, Falk MJ, Holmbeck MA, Manfredi G, Shadel GS, Vernon HJ, Zolkipli-Cunningham Z. Nutritional Interventions for Mitochondrial OXPHOS Deficiencies: Mechanisms and Model Systems. Annual Review of Pathology. PMID 29099651 DOI: 10.1146/Annurev-Pathol-020117-043644 |
0.408 |
|
| 2017 |
Valsecchi F, Konrad C, D'Aurelio M, Ramos-Espiritu LS, Stepanova A, Burstein SR, Galkin A, Magranè J, Starkov A, Buck J, Levin LR, Manfredi G. Distinct intracellular sAC-cAMP domains regulate ER calcium signaling and OXPHOS function. Journal of Cell Science. PMID 28864766 DOI: 10.1242/Jcs.206318 |
0.392 |
|
| 2017 |
Kenny TC, Manfredi G, Germain D. The Mitochondrial Unfolded Protein Response as a Non-Oncogene Addiction to Support Adaptation to Stress during Transformation in Cancer and Beyond. Frontiers in Oncology. 7: 159. PMID 28798902 DOI: 10.3389/Fonc.2017.00159 |
0.452 |
|
| 2017 |
Kahl A, Anderson CJ, Qian L, Voss H, Manfredi G, Iadecola C, Zhou P. Neuronal expression of the mitochondrial protein prohibitin confers profound neuroprotection in a mouse model of focal cerebral ischemia. Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism. 271678X17720371. PMID 28714328 DOI: 10.1177/0271678X17720371 |
0.397 |
|
| 2017 |
Kawamata H, Peixoto P, Konrad C, Palomo G, Bredvik K, Gerges M, Valsecchi F, Petrucelli L, Ravits JM, Starkov A, Manfredi G. Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo. Molecular Neurodegeneration. 12: 37. PMID 28482850 DOI: 10.1186/S13024-017-0180-1 |
0.802 |
|
| 2017 |
Kenny TC, Hart P, Ragazzi M, Sersinghe M, Chipuk J, Sagar MA, Eliceiri KW, LaFramboise T, Grandhi S, Santos J, Riar AK, Papa L, D'Aurello M, Manfredi G, Bonini MG, et al. Selected mitochondrial DNA landscapes activate the SIRT3 axis of the UPR(mt) to promote metastasis. Oncogene. PMID 28368421 DOI: 10.1038/Onc.2017.52 |
0.583 |
|
| 2017 |
Riar AK, Burstein SR, Palomo G, Arreguin A, Manfredi G, Germain D. Sex specific activation of the ERα axis of the mitochondrial UPR (UPRmt) in the G93A-SOD1 mouse model of familial ALS. Human Molecular Genetics. PMID 28186560 DOI: 10.1093/Hmg/Ddx049 |
0.479 |
|
| 2016 |
Stepanova A, Shurubor Y, Valsecchi F, Manfredi G, Galkin A. Differential susceptibility of mitochondrial complex II to inhibition by oxaloacetate in brain and heart. Biochimica Et Biophysica Acta. PMID 27287543 DOI: 10.1016/J.Bbabio.2016.06.002 |
0.355 |
|
| 2016 |
Doczi J, Torocsik B, Echaniz-Laguna A, Mousson de Camaret B, Starkov A, Starkova N, Gál A, Molnár MJ, Kawamata H, Manfredi G, Adam-Vizi V, Chinopoulos C. Alterations in voltage-sensing of the mitochondrial permeability transition pore in ANT1-deficient cells. Scientific Reports. 6: 26700. PMID 27221760 DOI: 10.1038/Srep26700 |
0.532 |
|
| 2016 |
Inan M, Zhao M, Manuszak M, Karakaya C, Rajadhyaksha A, Pickel VM, Schwartz TH, Goldstein PA, Manfredi G. Energy deficit in parvalbumin neurons leads to circuit dysfunction, impaired sensory gating and social disability. Neurobiology of Disease. PMID 27105708 DOI: 10.1016/J.Nbd.2016.04.004 |
0.455 |
|
| 2016 |
Cloonan SM, Glass K, Laucho-Contreras ME, Bhashyam AR, Cervo M, Pabón MA, Konrad C, Polverino F, Siempos II, Perez E, Mizumura K, Ghosh MC, Parameswaran H, Williams NC, Rooney KT, ... ... Manfredi G, et al. Mitochondrial iron chelation ameliorates cigarette smoke-induced bronchitis and emphysema in mice. Nature Medicine. PMID 26752519 DOI: 10.1038/Nm.4021 |
0.402 |
|
| 2016 |
Manfredi G, Kawamata H. Mitochondria and endoplasmic reticulum crosstalk in amyotrophic lateral sclerosis. Neurobiology of Disease. 90: 35-42. PMID 26282323 DOI: 10.1016/J.Nbd.2015.08.004 |
0.455 |
|
| 2015 |
Palomo GM, Manfredi G. Exploring new pathways of neurodegeneration in ALS: the role of mitochondria quality control. Brain Research. 1607: 36-46. PMID 25301687 DOI: 10.1016/J.Brainres.2014.09.065 |
0.549 |
|
| 2014 |
Kirk K, Gennings C, Hupf JC, Tadesse S, D'Aurelio M, Kawamata H, Valsecchi F, Mitsumoto H, Manfredi G. Bioenergetic markers in skin fibroblasts of sporadic amyotrophic lateral sclerosis and progressive lateral sclerosis patients. Annals of Neurology. 76: 620-4. PMID 25090982 DOI: 10.1002/Ana.24244 |
0.425 |
|
| 2014 |
Hess KC, Liu J, Manfredi G, Mühlschlegel FA, Buck J, Levin LR, Barrientos A. A mitochondrial CO2-adenylyl cyclase-cAMP signalosome controls yeast normoxic cytochrome c oxidase activity. Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology. 28: 4369-80. PMID 25002117 DOI: 10.1096/Fj.14-252890 |
0.441 |
|
| 2014 |
Papa L, Manfredi G, Germain D. SOD1, an unexpected novel target for cancer therapy. Genes & Cancer. 5: 15-21. PMID 24955214 DOI: 10.18632/Genesandcancer.4 |
0.438 |
|
| 2014 |
Valsecchi F, Konrad C, Manfredi G. Role of soluble adenylyl cyclase in mitochondria. Biochimica Et Biophysica Acta. 1842: 2555-60. PMID 24907564 DOI: 10.1016/J.Bbadis.2014.05.035 |
0.554 |
|
| 2014 |
Acín-Pérez R, Carrascoso I, Baixauli F, Roche-Molina M, Latorre-Pellicer A, Fernández-Silva P, Mittelbrunn M, Sanchez-Madrid F, Pérez-Martos A, Lowell CA, Manfredi G, Enríquez JA. ROS-triggered phosphorylation of complex II by Fgr kinase regulates cellular adaptation to fuel use. Cell Metabolism. 19: 1020-33. PMID 24856931 DOI: 10.1016/J.Cmet.2014.04.015 |
0.439 |
|
| 2014 |
Jackman KA, Zhou P, Faraco G, Peixoto PM, Coleman C, Voss HU, Pickel V, Manfredi G, Iadecola C. Dichotomous effects of chronic intermittent hypoxia on focal cerebral ischemic injury. Stroke; a Journal of Cerebral Circulation. 45: 1460-7. PMID 24713530 DOI: 10.1161/Strokeaha.114.004816 |
0.702 |
|
| 2014 |
Mishra P, Carelli V, Manfredi G, Chan DC. Proteolytic cleavage of Opa1 stimulates mitochondrial inner membrane fusion and couples fusion to oxidative phosphorylation. Cell Metabolism. 19: 630-41. PMID 24703695 DOI: 10.1016/J.Cmet.2014.03.011 |
0.497 |
|
| 2014 |
Kurinami H, Shimamura M, Ma T, Qian L, Koizumi K, Park L, Klann E, Manfredi G, Iadecola C, Zhou P. Prohibitin viral gene transfer protects hippocampal CA1 neurons from ischemia and ameliorates postischemic hippocampal dysfunction. Stroke; a Journal of Cerebral Circulation. 45: 1131-8. PMID 24619393 DOI: 10.1161/Strokeaha.113.003577 |
0.377 |
|
| 2014 |
Kawamata H, Ng SK, Diaz N, Burstein S, Morel L, Osgood A, Sider B, Higashimori H, Haydon PG, Manfredi G, Yang Y. Abnormal intracellular calcium signaling and SNARE-dependent exocytosis contributes to SOD1G93A astrocyte-mediated toxicity in amyotrophic lateral sclerosis. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 34: 2331-48. PMID 24501372 DOI: 10.1523/Jneurosci.2689-13.2014 |
0.315 |
|
| 2014 |
Magrané J, Cortez C, Gan WB, Manfredi G. Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models. Human Molecular Genetics. 23: 1413-24. PMID 24154542 DOI: 10.1093/Hmg/Ddt528 |
0.538 |
|
| 2014 |
Mishra P, Carelli V, Manfredi G, Chan DC. Erratum: Proteolytic cleavage of Opa1 stimulates mitochondrial inner membrane fusion and couples fusion to oxidative phosphorylation (Cell Metabolism (2014) 19 (630-641)) Cell Metabolism. 19. DOI: 10.1016/J.Cmet.2014.04.014 |
0.452 |
|
| 2013 |
Peixoto PM, Kim HJ, Sider B, Starkov A, Horvath TL, Manfredi G. UCP2 overexpression worsens mitochondrial dysfunction and accelerates disease progression in a mouse model of amyotrophic lateral sclerosis. Molecular and Cellular Neurosciences. 57: 104-10. PMID 24141050 DOI: 10.1016/J.Mcn.2013.10.002 |
0.79 |
|
| 2013 |
Turner MR, Bowser R, Bruijn L, Dupuis L, Ludolph A, McGrath M, Manfredi G, Maragakis N, Miller RG, Pullman SL, Rutkove SB, Shaw PJ, Shefner J, Fischbeck KH. Mechanisms, models and biomarkers in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 14: 19-32. PMID 23678877 DOI: 10.3109/21678421.2013.778554 |
0.362 |
|
| 2013 |
Valsecchi F, Ramos-Espiritu LS, Buck J, Levin LR, Manfredi G. cAMP and mitochondria. Physiology (Bethesda, Md.). 28: 199-209. PMID 23636265 DOI: 10.1152/Physiol.00004.2013 |
0.513 |
|
| 2013 |
Gonzalvez F, D'Aurelio M, Boutant M, Moustapha A, Puech JP, Landes T, Arnauné-Pelloquin L, Vial G, Taleux N, Slomianny C, Wanders RJ, Houtkooper RH, Bellenguer P, Møller IM, Gottlieb E, ... ... Manfredi G, et al. Barth syndrome: cellular compensation of mitochondrial dysfunction and apoptosis inhibition due to changes in cardiolipin remodeling linked to tafazzin (TAZ) gene mutation. Biochimica Et Biophysica Acta. 1832: 1194-206. PMID 23523468 DOI: 10.1016/J.Bbadis.2013.03.005 |
0.553 |
|
| 2013 |
Kiss G, Konrad C, Doczi J, Starkov AA, Kawamata H, Manfredi G, Zhang SF, Gibson GE, Beal MF, Adam-Vizi V, Chinopoulos C. The negative impact of α-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation. Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology. 27: 2392-406. PMID 23475850 DOI: 10.1096/Fj.12-220202 |
0.46 |
|
| 2012 |
Kim HJ, Magranè J, Starkov AA, Manfredi G. The mitochondrial calcium regulator cyclophilin D is an essential component of oestrogen-mediated neuroprotection in amyotrophic lateral sclerosis. Brain : a Journal of Neurology. 135: 2865-74. PMID 22961554 DOI: 10.1093/Brain/Aws208 |
0.46 |
|
| 2012 |
Gong J, Hoyos B, Acin-Perez R, Vinogradov V, Shabrova E, Zhao F, Leitges M, Fischman D, Manfredi G, Hammerling U. Two protein kinase C isoforms, δ and ε, regulate energy homeostasis in mitochondria by transmitting opposing signals to the pyruvate dehydrogenase complex. Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology. 26: 3537-49. PMID 22573912 DOI: 10.1096/Fj.11-197376 |
0.372 |
|
| 2012 |
Zhou P, Qian L, D'Aurelio M, Cho S, Wang G, Manfredi G, Pickel V, Iadecola C. Prohibitin reduces mitochondrial free radical production and protects brain cells from different injury modalities. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 32: 583-92. PMID 22238093 DOI: 10.1523/Jneurosci.2849-11.2012 |
0.522 |
|
| 2012 |
Magrané J, Sahawneh MA, Przedborski S, Estévez ÁG, Manfredi G. Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 32: 229-42. PMID 22219285 DOI: 10.1523/Jneurosci.1233-11.2012 |
0.464 |
|
| 2012 |
Hoyos B, Acin-Perez R, Fischman DA, Manfredi G, Hammerling U. Hiding in plain sight: uncovering a new function of vitamin A in redox signaling. Biochimica Et Biophysica Acta. 1821: 241-7. PMID 21763457 DOI: 10.1016/J.Bbalip.2011.06.014 |
0.33 |
|
| 2011 |
Igoudjil A, Magrané J, Fischer LR, Kim HJ, Hervias I, Dumont M, Cortez C, Glass JD, Starkov AA, Manfredi G. In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 31: 15826-37. PMID 22049426 DOI: 10.1523/Jneurosci.1965-11.2011 |
0.588 |
|
| 2011 |
Rak M, McStay GP, Fujikawa M, Yoshida M, Manfredi G, Tzagoloff A. Turnover of ATP synthase subunits in F 1-depleted HeLa and yeast cells Febs Letters. 585: 2582-2586. PMID 21784071 DOI: 10.1016/J.Febslet.2011.07.011 |
0.38 |
|
| 2011 |
Liu W, Acín-Peréz R, Geghman KD, Manfredi G, Lu B, Li C. Pink1 regulates the oxidative phosphorylation machinery via mitochondrial fission. Proceedings of the National Academy of Sciences of the United States of America. 108: 12920-4. PMID 21768365 DOI: 10.1073/Pnas.1107332108 |
0.602 |
|
| 2011 |
Mitsuhashi S, Hatakeyama H, Karahashi M, Koumura T, Nonaka I, Hayashi YK, Noguchi S, Sher RB, Nakagawa Y, Manfredi G, Goto Y, Cox GA, Nishino I. Muscle choline kinase beta defect causes mitochondrial dysfunction and increased mitophagy. Human Molecular Genetics. 20: 3841-51. PMID 21750112 DOI: 10.1093/Hmg/Ddr305 |
0.488 |
|
| 2011 |
Acin-Perez R, Russwurm M, Günnewig K, Gertz M, Zoidl G, Ramos L, Buck J, Levin LR, Rassow J, Manfredi G, Steegborn C. A phosphodiesterase 2A isoform localized to mitochondria regulates respiration. The Journal of Biological Chemistry. 286: 30423-32. PMID 21724846 DOI: 10.1074/Jbc.M111.266379 |
0.539 |
|
| 2011 |
Acin-Perez R, Gatti DL, Bai Y, Manfredi G. Protein phosphorylation and prevention of cytochrome oxidase inhibition by ATP: coupled mechanisms of energy metabolism regulation. Cell Metabolism. 13: 712-9. PMID 21641552 DOI: 10.1016/J.Cmet.2011.03.024 |
0.446 |
|
| 2011 |
Kawamata H, Tiranti V, Magrané J, Chinopoulos C, Manfredi G. adPEO mutations in ANT1 impair ADP-ATP translocation in muscle mitochondria. Human Molecular Genetics. 20: 2964-74. PMID 21586654 DOI: 10.1093/Hmg/Ddr200 |
0.541 |
|
| 2011 |
Fischer LR, Igoudjil A, Magrané J, Li Y, Hansen JM, Manfredi G, Glass JD. SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse. Brain : a Journal of Neurology. 134: 196-209. PMID 21078595 DOI: 10.1093/Brain/Awq314 |
0.471 |
|
| 2011 |
Acin-Perez⁎ R, Manfredi G. Regulation of oxidative phosphorylation through phosphorylation of cytochrome oxidase Mitochondrion. 11: 655. DOI: 10.1016/J.Mito.2011.03.061 |
0.312 |
|
| 2011 |
D'Aurelio⁎ M, Yang L, Chen Q, Beal F, Gross S, Manfredi G. Impaired amino acid metabolism in cells affected by the T8993G (NARP) mtDNA mutation: A potential novel therapeutic target Mitochondrion. 11: 652. DOI: 10.1016/J.Mito.2011.03.053 |
0.378 |
|
| 2010 |
Escobar-Alvarez S, Gardner J, Sheth A, Manfredi G, Yang G, Ouerfelli O, Heaney ML, Scheinberg DA. Inhibition of human peptide deformylase disrupts mitochondrial function. Molecular and Cellular Biology. 30: 5099-109. PMID 20805355 DOI: 10.1128/Mcb.00469-10 |
0.509 |
|
| 2010 |
Acin-Perez R, Hoyos B, Gong J, Vinogradov V, Fischman DA, Leitges M, Borhan B, Starkov A, Manfredi G, Hammerling U. Regulation of intermediary metabolism by the PKCdelta signalosome in mitochondria. Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology. 24: 5033-42. PMID 20798245 DOI: 10.1096/fj.10.166934 |
0.376 |
|
| 2010 |
Kawamata H, Starkov AA, Manfredi G, Chinopoulos C. A kinetic assay of mitochondrial ADP-ATP exchange rate in permeabilized cells. Analytical Biochemistry. 407: 52-7. PMID 20691655 DOI: 10.1016/J.Ab.2010.07.031 |
0.449 |
|
| 2010 |
Suen DF, Narendra DP, Tanaka A, Manfredi G, Youle RJ. Parkin overexpression selects against a deleterious mtDNA mutation in heteroplasmic cybrid cells. Proceedings of the National Academy of Sciences of the United States of America. 107: 11835-40. PMID 20547844 DOI: 10.1073/Pnas.0914569107 |
0.596 |
|
| 2010 |
Kawamata H, Manfredi G. Mitochondrial dysfunction and intracellular calcium dysregulation in ALS. Mechanisms of Ageing and Development. 131: 517-26. PMID 20493207 DOI: 10.1016/J.Mad.2010.05.003 |
0.499 |
|
| 2010 |
Kawamata H, Manfredi G. Import, maturation, and function of SOD1 and its copper chaperone CCS in the mitochondrial intermembrane space. Antioxidants & Redox Signaling. 13: 1375-84. PMID 20367259 DOI: 10.1089/Ars.2010.3212 |
0.561 |
|
| 2010 |
Vives-Bauza C, Magrané J, Andreu AL, Manfredi G. Novel role of ATPase subunit C targeting peptides beyond mitochondrial protein import. Molecular Biology of the Cell. 21: 131-9. PMID 19889836 DOI: 10.1091/Mbc.E09-06-0483 |
0.422 |
|
| 2010 |
D'Aurelio M, Vives-Bauza C, Davidson MM, Manfredi G. Mitochondrial DNA background modifies the bioenergetics of NARP/MILS ATP6 mutant cells. Human Molecular Genetics. 19: 374-86. PMID 19875463 DOI: 10.1093/Hmg/Ddp503 |
0.55 |
|
| 2010 |
Yang H, Brosel S, Acin-Perez R, Slavkovich V, Nishino I, Khan R, Goldberg IJ, Graziano J, Manfredi G, Schon EA. Analysis of mouse models of cytochrome c oxidase deficiency owing to mutations in Sco2. Human Molecular Genetics. 19: 170-80. PMID 19837698 DOI: 10.1093/Hmg/Ddp477 |
0.5 |
|
| 2010 |
Acin-Perez R, Hoyos B, Zhao F, Vinogradov V, Fischman DA, Harris RA, Leitges M, Wongsiriroj N, Blaner WS, Manfredi G, Hammerling U. Control of oxidative phosphorylation by vitamin A illuminates a fundamental role in mitochondrial energy homoeostasis. Faseb Journal : Official Publication of the Federation of American Societies For Experimental Biology. 24: 627-36. PMID 19812372 DOI: 10.1096/Fj.09-142281 |
0.405 |
|
| 2009 |
Acin-Perez R, Salazar E, Brosel S, Yang H, Schon EA, Manfredi G. Modulation of mitochondrial protein phosphorylation by soluble adenylyl cyclase ameliorates cytochrome oxidase defects. Embo Molecular Medicine. 1: 392-406. PMID 20049744 DOI: 10.1002/Emmm.200900046 |
0.581 |
|
| 2009 |
Magrané J, Hervias I, Henning MS, Damiano M, Kawamata H, Manfredi G. Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities. Human Molecular Genetics. 18: 4552-64. PMID 19779023 DOI: 10.1093/Hmg/Ddp421 |
0.547 |
|
| 2009 |
Magrané J, Manfredi G. Mitochondrial function, morphology, and axonal transport in amyotrophic lateral sclerosis. Antioxidants & Redox Signaling. 11: 1615-26. PMID 19344253 DOI: 10.1089/Ars.2009.2604 |
0.409 |
|
| 2009 |
Acin-Perez R, Salazar E, Kamenetsky M, Buck J, Levin LR, Manfredi G. Cyclic AMP produced inside mitochondria regulates oxidative phosphorylation. Cell Metabolism. 9: 265-76. PMID 19254571 DOI: 10.1016/J.Cmet.2009.01.012 |
0.489 |
|
| 2009 |
Liu W, Vives-Bauza C, AcÃn-Peréz- R, Yamamoto A, Tan Y, Li Y, Magrané J, Stavarache MA, Shaffer S, Chang S, Kaplitt MG, Huang XY, Beal MF, Manfredi G, Li C. PINK1 defect causes mitochondrial dysfunction, proteasomal deficit and alpha-synuclein aggregation in cell culture models of Parkinson's disease. Plos One. 4: e4597. PMID 19242547 DOI: 10.1371/Journal.Pone.0004597 |
0.557 |
|
| 2008 |
Kawamata H, Magrané J, Kunst C, King MP, Manfredi G. Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria. The Journal of Biological Chemistry. 283: 28321-8. PMID 18715867 DOI: 10.1074/Jbc.M805599200 |
0.591 |
|
| 2008 |
Kawamata H, Manfredi G. Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria. Human Molecular Genetics. 17: 3303-17. PMID 18703498 DOI: 10.1093/Hmg/Ddn226 |
0.566 |
|
| 2008 |
Vives-Bauza C, Anand M, Shiraz AK, Shirazi AK, Magrane J, Gao J, Vollmer-Snarr HR, Manfredi G, Finnemann SC. The age lipid A2E and mitochondrial dysfunction synergistically impair phagocytosis by retinal pigment epithelial cells. The Journal of Biological Chemistry. 283: 24770-80. PMID 18621729 DOI: 10.1074/Jbc.M800706200 |
0.528 |
|
| 2007 |
Kwong JQ, Henning MS, Starkov AA, Manfredi G. The mitochondrial respiratory chain is a modulator of apoptosis. The Journal of Cell Biology. 179: 1163-77. PMID 18086914 DOI: 10.1083/Jcb.200704059 |
0.774 |
|
| 2007 |
Manfredi G, Beal MF. Merging mitochondria for neuronal survival. Nature Medicine. 13: 1140-1. PMID 17917656 DOI: 10.1038/Nm1007-1140 |
0.335 |
|
| 2007 |
Li Y, D'Aurelio M, Deng JH, Park JS, Manfredi G, Hu P, Lu J, Bai Y. An assembled complex IV maintains the stability and activity of complex I in mammalian mitochondria. The Journal of Biological Chemistry. 282: 17557-62. PMID 17452320 DOI: 10.1074/Jbc.M701056200 |
0.372 |
|
| 2007 |
Bell CJ, Manfredi G, Griffiths EJ, Rutter GA. Luciferase expression for ATP imaging: application to cardiac myocytes. Methods in Cell Biology. 80: 341-52. PMID 17445703 DOI: 10.1016/S0091-679X(06)80017-8 |
0.362 |
|
| 2007 |
Vives-Bauza C, Yang L, Manfredi G. Assay of mitochondrial ATP synthesis in animal cells and tissues. Methods in Cell Biology. 80: 155-71. PMID 17445693 DOI: 10.1016/S0091-679X(06)80007-5 |
0.489 |
|
| 2007 |
Son M, Puttaparthi K, Kawamata H, Rajendran B, Boyer PJ, Manfredi G, Elliott JL. Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology. Proceedings of the National Academy of Sciences of the United States of America. 104: 6072-7. PMID 17389365 DOI: 10.1073/Pnas.0610923104 |
0.386 |
|
| 2006 |
Petri S, Kiaei M, Damiano M, Hiller A, Wille E, Manfredi G, Calingasan NY, Szeto HH, Beal MF. Cell-permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis. Journal of Neurochemistry. 98: 1141-8. PMID 16895581 DOI: 10.1111/J.1471-4159.2006.04018.X |
0.467 |
|
| 2006 |
Kwong JQ, Beal MF, Manfredi G. The role of mitochondria in inherited neurodegenerative diseases. Journal of Neurochemistry. 97: 1659-75. PMID 16805775 DOI: 10.1111/J.1471-4159.2006.03990.X |
0.762 |
|
| 2006 |
D'Aurelio M, Gajewski CD, Lenaz G, Manfredi G. Respiratory chain supercomplexes set the threshold for respiration defects in human mtDNA mutant cybrids. Human Molecular Genetics. 15: 2157-69. PMID 16740593 DOI: 10.1093/Hmg/Ddl141 |
0.601 |
|
| 2006 |
Manfredi G. mtDNA clock runs out for dopaminergic neurons. Nature Genetics. 38: 507-8. PMID 16642013 DOI: 10.1038/Ng0506-507 |
0.441 |
|
| 2006 |
Cassandrini D, Calevo MG, Tessa A, Manfredi G, Fattori F, Meschini MC, Carrozzo R, Tonoli E, Pedemonte M, Minetti C, Zara F, Santorelli FM, Bruno C. A new method for analysis of mitochondrial DNA point mutations and assess levels of heteroplasmy. Biochemical and Biophysical Research Communications. 342: 387-93. PMID 16483543 DOI: 10.1016/J.Bbrc.2006.01.152 |
0.518 |
|
| 2006 |
Damiano M, Starkov AA, Petri S, Kipiani K, Kiaei M, Mattiazzi M, Flint Beal M, Manfredi G. Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-superoxide dismutase mutant mice. Journal of Neurochemistry. 96: 1349-61. PMID 16478527 DOI: 10.1111/J.1471-4159.2006.03619.X |
0.486 |
|
| 2006 |
Hervias I, Beal MF, Manfredi G. Mitochondrial dysfunction and amyotrophic lateral sclerosis. Muscle & Nerve. 33: 598-608. PMID 16372325 DOI: 10.1002/Mus.20489 |
0.578 |
|
| 2006 |
Vives-Bauza C, Gonzalo R, Manfredi G, Garcia-Arumi E, Andreu AL. Enhanced ROS production and antioxidant defenses in cybrids harbouring mutations in mtDNA. Neuroscience Letters. 391: 136-41. PMID 16165271 DOI: 10.1016/J.Neulet.2005.08.049 |
0.563 |
|
| 2006 |
Kawamata H, Hervias I, Kiaei M, King M, Kunst C, Manfredi G. Lysyl-tRNA synthetase: A target for mutant SOD1 toxicity in mitochondria Mitochondrion. 6: 271-272. DOI: 10.1016/J.Mito.2006.08.021 |
0.444 |
|
| 2005 |
Manfredi G, Xu Z. Mitochondrial dysfunction and its role in motor neuron degeneration in ALS. Mitochondrion. 5: 77-87. PMID 16050975 DOI: 10.1016/J.Mito.2005.01.002 |
0.598 |
|
| 2005 |
Vijayvergiya C, Beal MF, Buck J, Manfredi G. Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 25: 2463-70. PMID 15758154 DOI: 10.1523/Jneurosci.4385-04.2005 |
0.782 |
|
| 2005 |
Shidara Y, Yamagata K, Kanamori T, Nakano K, Kwong JQ, Manfredi G, Oda H, Ohta S. Positive contribution of pathogenic mutations in the mitochondrial genome to the promotion of cancer by prevention from apoptosis. Cancer Research. 65: 1655-63. PMID 15753359 DOI: 10.1158/0008-5472.Can-04-2012 |
0.764 |
|
| 2004 |
D'Aurelio M, Gajewski CD, Lin MT, Mauck WM, Shao LZ, Lenaz G, Moraes CT, Manfredi G. Heterologous mitochondrial DNA recombination in human cells. Human Molecular Genetics. 13: 3171-9. PMID 15496432 DOI: 10.1093/Hmg/Ddh326 |
0.417 |
|
| 2004 |
Mattiazzi M, Vijayvergiya C, Gajewski CD, DeVivo DC, Lenaz G, Wiedmann M, Manfredi G. The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants. Human Molecular Genetics. 13: 869-79. PMID 14998933 DOI: 10.1093/Hmg/Ddh103 |
0.762 |
|
| 2003 |
Gajewski CD, Yang L, Schon EA, Manfredi G. New insights into the bioenergetics of mitochondrial disorders using intracellular ATP reporters. Molecular Biology of the Cell. 14: 3628-35. PMID 12972552 DOI: 10.1091/Mbc.E02-12-0796 |
0.566 |
|
| 2003 |
Bayona-Bafaluy MP, Manfredi G, Moraes CT. A chemical enucleation method for the transfer of mitochondrial DNA to rho(o) cells. Nucleic Acids Research. 31: e98. PMID 12907750 DOI: 10.1093/Nar/Gng100 |
0.485 |
|
| 2003 |
Barrientos A, Korr D, Barwell KJ, Sjulsen C, Gajewski CD, Manfredi G, Ackerman S, Tzagoloff A. MTG1 codes for a conserved protein required for mitochondrial translation Molecular Biology of the Cell. 14: 2292-2302. PMID 12808030 DOI: 10.1091/Mbc.E02-10-0636 |
0.558 |
|
| 2003 |
Gajewski CD, Lin MT, Cudkowicz ME, Beal MF, Manfredi G. Mitochondrial DNA from platelets of sporadic ALS patients restores normal respiratory functions in rho(0) cells. Experimental Neurology. 179: 229-35. PMID 12618129 DOI: 10.1016/S0014-4886(02)00022-5 |
0.528 |
|
| 2003 |
Schon EA, Manfredi G. Neuronal degeneration and mitochondrial dysfunction. The Journal of Clinical Investigation. 111: 303-12. PMID 12569152 DOI: 10.1172/Jci17741 |
0.59 |
|
| 2003 |
Manfredi G, Kwong JQ, Oca-Cossio JA, Woischnik M, Gajewski CD, Martushova K, D'Aurelio M, Friedlich AL, Moraes CT. BCL-2 improves oxidative phosphorylation and modulates adenine nucleotide translocation in mitochondria of cells harboring mutant mtDNA. The Journal of Biological Chemistry. 278: 5639-45. PMID 12431997 DOI: 10.1074/Jbc.M203080200 |
0.795 |
|
| 2002 |
Guy J, Qi X, Pallotti F, Schon EA, Manfredi G, Carelli V, Martinuzzi A, Hauswirth WW, Lewin AS. Rescue of a mitochondrial deficiency causing Leber Hereditary Optic Neuropathy. Annals of Neurology. 52: 534-42. PMID 12402249 DOI: 10.1002/Ana.10354 |
0.534 |
|
| 2002 |
Chirichigno JW, Manfredi G, Beal MF, Albers DS. Stress-induced mitochondrial depolarization and oxidative damage in PSP cybrids. Brain Research. 951: 31-5. PMID 12231453 DOI: 10.1016/S0006-8993(02)03101-3 |
0.569 |
|
| 2002 |
Manfredi G, Yang L, Gajewski CD, Mattiazzi M. Measurements of ATP in mammalian cells. Methods (San Diego, Calif.). 26: 317-26. PMID 12054922 DOI: 10.1016/S1046-2023(02)00037-3 |
0.446 |
|
| 2002 |
Mattiazzi M, D'Aurelio M, Gajewski CD, Martushova K, Kiaei M, Beal MF, Manfredi G. Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. The Journal of Biological Chemistry. 277: 29626-33. PMID 12050154 DOI: 10.1074/Jbc.M203065200 |
0.603 |
|
| 2002 |
Manfredi G, Fu J, Ojaimi J, Sadlock JE, Kwong JQ, Guy J, Schon EA. Rescue of a deficiency in ATP synthesis by transfer of MTATP6, a mitochondrial DNA-encoded gene, to the nucleus. Nature Genetics. 30: 394-9. PMID 11925565 DOI: 10.1038/Ng851 |
0.774 |
|
| 2002 |
Wiedemann FR, Manfredi G, Mawrin C, Beal MF, Schon EA. Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients. Journal of Neurochemistry. 80: 616-25. PMID 11841569 DOI: 10.1046/J.0022-3042.2001.00731.X |
0.476 |
|
| 2002 |
Vives-Bauza C, Andreu AL, Manfredi G, Beal MF, Janetzky B, Gruenewald TH, Lin MT. Sequence analysis of the entire mitochondrial genome in Parkinson's disease. Biochemical and Biophysical Research Communications. 290: 1593-601. PMID 11820805 DOI: 10.1006/Bbrc.2002.6388 |
0.404 |
|
| 2001 |
Manfredi G, Kwong JQ, Oca-Cossio JA, Aurelio MD, Gajewsky CD, Beal FM, Moraes CT. Bcl-2 Suppresses Oxidative Phosphorylation Defects Caused by Mitochondrial DNA Mutations. Thescientificworldjournal. 1: 39. PMID 30147485 DOI: 10.1100/Tsw.2001.23.144 |
0.799 |
|
| 2001 |
D'Aurelio M, Pallotti F, Barrientos A, Gajewski CD, Kwong JQ, Bruno C, Beal MF, Manfredi G. In vivo regulation of oxidative phosphorylation in cells harboring a stop-codon mutation in mitochondrial DNA-encoded cytochrome c oxidase subunit I. The Journal of Biological Chemistry. 276: 46925-32. PMID 11595737 DOI: 10.1074/Jbc.M106429200 |
0.751 |
|
| 2001 |
Manfredi G, Spinazzola A, Checcarelli N, Naini A. Assay of mitochondrial ATP synthesis in animal cells. Methods in Cell Biology. 65: 133-45. PMID 11381590 DOI: 10.1016/S0091-679X(01)65008-8 |
0.591 |
|
| 2001 |
Albers DS, Swerdlow RH, Manfredi G, Gajewski C, Yang L, Parker WD, Beal MF. Further evidence for mitochondrial dysfunction in progressive supranuclear palsy. Experimental Neurology. 168: 196-8. PMID 11170735 DOI: 10.1006/Exnr.2000.7607 |
0.586 |
|
| 2000 |
Tang Y, Manfredi G, Hirano M, Schon EA. Maintenance of human rearranged mitochondrial DNAs in long-term cultured transmitochondrial cell lines. Molecular Biology of the Cell. 11: 2349-58. PMID 10888673 DOI: 10.1091/Mbc.11.7.2349 |
0.516 |
|
| 2000 |
Manfredi G, Beal MF. The role of mitochondria in the pathogenesis of neurodegenerative diseases. Brain Pathology (Zurich, Switzerland). 10: 462-72. PMID 10885665 DOI: 10.1111/J.1750-3639.2000.Tb00278.X |
0.538 |
|
| 1999 |
Bruno C, Martinuzzi A, Tang Y, Andreu AL, Pallotti F, Bonilla E, Shanske S, Fu J, Sue CM, Angelini C, DiMauro S, Manfredi G. A stop-codon mutation in the human mtDNA cytochrome c oxidase I gene disrupts the functional structure of complex IV. American Journal of Human Genetics. 65: 611-20. PMID 10441567 DOI: 10.1086/302546 |
0.511 |
|
| 1999 |
Manfredi G, Gupta N, Vazquez-Memije ME, Sadlock JE, Spinazzola A, De Vivo DC, Schon EA. Oligomycin induces a decrease in the cellular content of a pathogenic mutation in the human mitochondrial ATPase 6 gene. The Journal of Biological Chemistry. 274: 9386-91. PMID 10092618 DOI: 10.1074/Jbc.274.14.9386 |
0.544 |
|
| 1998 |
Bruno C, Manfredi G, Andreu AL, Shanske S, Krishna S, Ilse WK, DiMauro S. A splice junction mutation in the alpha(M) gene of phosphorylase kinase in a patient with myopathy. Biochemical and Biophysical Research Communications. 249: 648-51. PMID 9731190 DOI: 10.1006/Bbrc.1998.9211 |
0.324 |
|
| 1997 |
Manfredi G, Thyagarajan D, Papadopoulou LC, Pallotti F, Schon EA. The fate of human sperm-derived mtDNA in somatic cells. American Journal of Human Genetics. 61: 953-60. PMID 9382109 DOI: 10.1086/514887 |
0.445 |
|
| 1997 |
Manfredi G, Vu T, Bonilla E, Schon EA, DiMauro S, Arnaudo E, Zhang L, Rowland LP, Hirano M. Association of myopathy with large-scale mitochondrial DNA duplications and deletions: which is pathogenic? Annals of Neurology. 42: 180-8. PMID 9266727 DOI: 10.1002/Ana.410420208 |
0.402 |
|
| 1997 |
Di Lazzaro V, Restuccia D, Servidei S, Valeriani M, Nardone R, Manfredi G, Silvestri G, Ricci E, Tonali P. Functional involvement of central nervous system in mitochondrial disorders Electroencephalography and Clinical Neurophysiology - Electromyography and Motor Control. 105: 171-180. PMID 9216485 DOI: 10.1016/S0924-980X(97)96671-6 |
0.474 |
|
| 1997 |
Hao H, Manfredi G, Moraes CT. Functional and structural features of a tandem duplication of the human mtDNA promoter region American Journal of Human Genetics. 60: 1363-1372. PMID 9199557 DOI: 10.1086/515474 |
0.429 |
|
| 1996 |
Massa R, Lodi R, Barbiroli B, Servidei S, Sancesario G, Manfredi G, Zaniol P, Bernardi G. Partial block of glycolysis in late-onset phosphofructokinase deficiency myopathy. Acta Neuropathologica. 91: 322-9. PMID 8834546 DOI: 10.1007/S004010050432 |
0.342 |
|
| 1996 |
Manfredi G, Schon EA, Bonilla E, Moraes CT, Shanske S, DiMauro S. Identification of a mutation in the mitochondrial tRNACys gene associated with mitochondrial encephalopathy Human Mutation. 7: 158-163. PMID 8829635 DOI: 10.1002/(Sici)1098-1004(1996)7:2<158::Aid-Humu12>3.0.Co;2-1 |
0.583 |
|
| 1996 |
Fromenty B, Manfredi G, Sadlock J, Zhang L, King MP, Schon EA. Efficient and specific amplification of identified partial duplications of human mitochondrial DNA by long PCR. Biochimica Et Biophysica Acta. 1308: 222-30. PMID 8809114 DOI: 10.1016/0167-4781(96)00110-8 |
0.443 |
|
| 1995 |
Manfredi G, Servidei S, Bonilla E, Shanske S, Schon EA, Dimauro S, Moraes CT. High levels of mitochondrial dna with an unstable 260-bp duplication in a patient with a mitochondrial myopathy Neurology. 45: 762-768. PMID 7723967 DOI: 10.1212/Wnl.45.4.762 |
0.514 |
|
| 1995 |
Manfredi G, Schon EA, Moraes CT, Bonilla E, Berry GT, Sladky JT, Dimauro S. A new mutation associated with MELAS is located in a mitochondrial DNA polypeptide-coding gene Neuromuscular Disorders. 5: 391-398. PMID 7496173 DOI: 10.1016/0960-8966(94)00079-O |
0.509 |
|
| 1994 |
Uncini A, Servidei S, Silvestri G, Manfredi G, Sabatelli M, Di Muzio A, Ricci E, Mirabella M, Di Mauro S, Tonali P. Ophthalmoplegia, demyelinating neuropathy, leukoencephalopathy, myopathy, and gastrointestinal dysfunction with multiple deletions of mitochondrial DNA: a mitochondrial multisystem disorder in search of a name. Muscle & Nerve. 17: 667-74. PMID 8196710 DOI: 10.1002/mus.880170616 |
0.445 |
|
| 1992 |
Mazziotta MRM, Ricci E, Bertini E, Vici CD, Servidei S, Burlina AB, Sabetta G, Bartuli A, Manfredi G, Silvestri G, Moraes CT, DiMauro S. Fatal infantile liver failure associated with mitochondrial DNA depletion The Journal of Pediatrics. 121: 896-901. PMID 1447652 DOI: 10.1016/S0022-3476(05)80335-X |
0.403 |
|
| 1991 |
Servidei S, Zeviani M, Manfredi G, Ricci E, Silvestri G, Bertini E, Gellera C, Di Mauro S, Di Donato S, Tonali P. Dominantly inherited mitochondrial myopathy with multiple deletions of mitochondrial DNA: clinical, morphologic, and biochemical studies. Neurology. 41: 1053-9. PMID 2067633 DOI: 10.1212/Wnl.41.7.1053 |
0.563 |
|
| 1991 |
Ciafaloni E, Ricci E, Servidei S, Shanske S, Silvestri G, Manfredi G, Schon EA, DiMauro S. Widespread tissue distribution of a tRNALeu(UUR) mutation in the mitochondrial DNA of a patient with MELAS syndrome. Neurology. 41: 1663-4. PMID 1922812 DOI: 10.1212/Wnl.41.10.1663 |
0.478 |
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