Mercedes Prudencio, Ph.D. - Publications

Affiliations: 
Mayo Clinic Jacksonville, Jacksonville, FL, United States 

33 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2019 Goodman LD, Prudencio M, Kramer NJ, Martinez-Ramirez LF, Srinivasan AR, Lan M, Parisi MJ, Zhu Y, Chew J, Cook CN, Berson A, Gitler AD, Petrucelli L, Bonini NM. Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD. Nature Neuroscience. PMID 31110321 DOI: 10.1038/s41593-019-0396-1  0.52
2019 Goodman LD, Prudencio M, Srinivasan AR, Rifai OM, Lee VM, Petrucelli L, Bonini NM. eIF4B and eIF4H mediate GR production from expanded G4C2 in a Drosophila model for C9orf72-associated ALS. Acta Neuropathologica Communications. 7: 62. PMID 31023341 DOI: 10.1186/s40478-019-0711-9  0.48
2019 Zhang YJ, Guo L, Gonzales PK, Gendron TF, Wu Y, Jansen-West K, O'Raw AD, Pickles SR, Prudencio M, Carlomagno Y, Gachechiladze MA, Ludwig C, Tian R, Chew J, DeTure M, et al. Heterochromatin anomalies and double-stranded RNA accumulation underlie poly(PR) toxicity. Science (New York, N.Y.). 363. PMID 30765536 DOI: 10.1126/science.aav2606  0.52
2018 Hanna Al Shaikh R, Caulfield T, Strongosky AJ, Matthew M, Jansen-West KR, Prudencio M, Fryer JD, Petrucelli L, Uitti RJ, Wszolek ZK. TRIO gene segregation in a family with cerebellar ataxia. Neurologia I Neurochirurgia Polska. PMID 30279051 DOI: 10.1016/j.pjnns.2018.09.006  0.52
2018 Ebbert MTW, Farrugia SL, Sens JP, Jansen-West K, Gendron TF, Prudencio M, McLaughlin IJ, Bowman B, Seetin M, DeJesus-Hernandez M, Jackson J, Brown PH, Dickson DW, van Blitterswijk M, Rademakers R, et al. Long-read sequencing across the C9orf72 'GGGGCC' repeat expansion: implications for clinical use and genetic discovery efforts in human disease. Molecular Neurodegeneration. 13: 46. PMID 30126445 DOI: 10.1186/s13024-018-0274-4  0.52
2018 Mordes DA, Prudencio M, Goodman LD, Klim JR, Moccia R, Limone F, Pietilainen O, Chowdhary K, Dickson DW, Rademakers R, Bonini NM, Petrucelli L, Eggan K. Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients. Acta Neuropathologica Communications. 6: 55. PMID 29973287 DOI: 10.1186/s40478-018-0555-8  0.52
2018 Zhang YJ, Gendron TF, Ebbert MTW, O'Raw AD, Yue M, Jansen-West K, Zhang X, Prudencio M, Chew J, Cook CN, Daughrity LM, Tong J, Song Y, Pickles SR, Castanedes-Casey M, et al. Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis. Nature Medicine. PMID 29942091 DOI: 10.1038/s41591-018-0071-1  0.52
2017 Lee CW, Stankowski JN, Chew J, Cook CN, Lam YW, Almeida S, Carlomagno Y, Lau KF, Prudencio M, Gao FB, Bogyo M, Dickson DW, Petrucelli L. The lysosomal protein cathepsin L is a progranulin protease. Molecular Neurodegeneration. 12: 55. PMID 28743268 DOI: 10.1186/s13024-017-0196-6  0.52
2017 Prudencio M, Gonzales PK, Cook CN, Gendron TF, Daughrity LM, Song Y, Ebbert MTW, van Blitterswijk M, Zhang YJ, Jansen-West K, Baker MC, DeTure M, Rademakers R, Boylan KB, Dickson DW, et al. Repetitive element transcripts are elevated in the brain of C9orf72 ALS/FTLD patients. Human Molecular Genetics. PMID 28637276 DOI: 10.1093/hmg/ddx233  0.52
2017 Gendron TF, Chew J, Stankowski JN, Hayes LR, Zhang YJ, Prudencio M, Carlomagno Y, Daughrity LM, Jansen-West K, Perkerson EA, O'Raw A, Cook C, Pregent L, Belzil V, van Blitterswijk M, et al. Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis. Science Translational Medicine. 9. PMID 28356511 DOI: 10.1126/scitranslmed.aai7866  0.52
2017 Moujalled D, Grubman A, Acevedo K, Yang S, Ke YD, Moujalled DM, Duncan C, Caragounis A, Perera ND, Turner BJ, Prudencio M, Petrucelli L, Blair I, Ittner LM, Crouch PJ, et al. TDP-43 mutations causing amyotrophic lateral sclerosis are associated with altered expression of RNA-binding protein hnRNP K and affect the Nrf2 antioxidant pathway. Human Molecular Genetics. PMID 28334913 DOI: 10.1093/hmg/ddx093  0.48
2016 Kramer NJ, Carlomagno Y, Zhang YJ, Almeida S, Cook CN, Gendron TF, Prudencio M, Van Blitterswijk M, Belzil V, Couthouis J, Paul JW, Goodman LD, Daughrity L, Chew J, Garrett A, et al. Spt4 selectively regulates the expression of C9orf72 sense and antisense mutant transcripts. Science (New York, N.Y.). 353: 708-12. PMID 27516603 DOI: 10.1126/science.aaf7791  1
2015 Mohagheghi F, Prudencio M, Stuani C, Cook C, Jansen-West K, Dickson DW, Petrucelli L, Buratti E. TDP-43 functions within a network of hnRNP proteins to inhibit the production of a truncated human SORT1 receptor. Human Molecular Genetics. PMID 26614389 DOI: 10.1093/hmg/ddv491  1
2015 Prudencio M, Belzil VV, Batra R, Ross CA, Gendron TF, Pregent LJ, Murray ME, Overstreet KK, Piazza-Johnston AE, Desaro P, Bieniek KF, DeTure M, Lee WC, Biendarra SM, Davis MD, et al. Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS. Nature Neuroscience. 18: 1175-82. PMID 26192745 DOI: 10.1038/nn.4065  1
2015 Chew J, Gendron TF, Prudencio M, Sasaguri H, Zhang YJ, Castanedes-Casey M, Lee CW, Jansen-West K, Kurti A, Murray ME, Bieniek KF, Bauer PO, Whitelaw EC, Rousseau L, Stankowski JN, et al. Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits. Science (New York, N.Y.). 348: 1151-4. PMID 25977373 DOI: 10.1126/science.aaa9344  0.52
2015 Chew J, Gendron TF, Prudencio M, Sasaguri H, Zhang YJ, Castanedes-Casey M, Lee CW, Jansen-West K, Kurti A, Murray ME, Bieniek KF, Bauer PO, Whitelaw EC, Rousseau L, Stankowski JN, et al. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits Science. 348: 1151-1154. DOI: 10.1126/science.aaa9344  1
2014 Zhang YJ, Jansen-West K, Xu YF, Gendron TF, Bieniek KF, Lin WL, Sasaguri H, Caulfield T, Hubbard J, Daughrity L, Chew J, Belzil VV, Prudencio M, Stankowski JN, Castanedes-Casey M, et al. Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress. Acta Neuropathologica. 128: 505-24. PMID 25173361 DOI: 10.1007/s00401-014-1336-5  1
2014 Lee WC, Almeida S, Prudencio M, Caulfield TR, Zhang YJ, Tay WM, Bauer PO, Chew J, Sasaguri H, Jansen-West KR, Gendron TF, Stetler CT, Finch N, Mackenzie IR, Rademakers R, et al. Targeted manipulation of the sortilin-progranulin axis rescues progranulin haploinsufficiency. Human Molecular Genetics. 23: 1467-78. PMID 24163244 DOI: 10.1093/hmg/ddt534  1
2014 Ayers J, Lelie H, Workman A, Prudencio M, Brown H, Fromholt S, Valentine J, Whitelegge J, Borchelt D. Distinctive features of the D101N and D101G variants of superoxide dismutase 1; two mutations that produce rapidly progressing motor neuron disease. Journal of Neurochemistry. 128: 305-14. PMID 24032979 DOI: 10.1111/jnc.12451  1
2013 Qualls DA, Prudencio M, Roberts BL, Crosby K, Brown H, Borchelt DR. Features of wild-type human SOD1 limit interactions with misfolded aggregates of mouse G86R Sod1. Molecular Neurodegeneration. 8: 46. PMID 24341866 DOI: 10.1186/1750-1326-8-46  1
2013 Belzil VV, Bauer PO, Prudencio M, Gendron TF, Stetler CT, Yan IK, Pregent L, Daughrity L, Baker MC, Rademakers R, Boylan K, Patel TC, Dickson DW, Petrucelli L. Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood. Acta Neuropathologica. 126: 895-905. PMID 24166615 DOI: 10.1007/s00401-013-1199-1  1
2013 Xu YF, Prudencio M, Hubbard JM, Tong J, Whitelaw EC, Jansen-West K, Stetler C, Cao X, Song J, Zhang YJ. The pathological phenotypes of human TDP-43 transgenic mouse models are independent of downregulation of mouse Tdp-43. Plos One. 8: e69864. PMID 23922830 DOI: 10.1371/journal.pone.0069864  1
2012 Prudencio M, Jansen-West KR, Lee WC, Gendron TF, Zhang YJ, Xu YF, Gass J, Stuani C, Stetler C, Rademakers R, Dickson DW, Buratti E, Petrucelli L. Misregulation of human sortilin splicing leads to the generation of a nonfunctional progranulin receptor. Proceedings of the National Academy of Sciences of the United States of America. 109: 21510-5. PMID 23236149 DOI: 10.1073/pnas.1211577110  1
2012 Gass J, Prudencio M, Stetler C, Petrucelli L. Progranulin: an emerging target for FTLD therapies. Brain Research. 1462: 118-28. PMID 22338605 DOI: 10.1016/j.brainres.2012.01.047  1
2012 Prudencio M, Lelie H, Brown HH, Whitelegge JP, Valentine JS, Borchelt DR. A novel variant of human superoxide dismutase 1 harboring amyotrophic lateral sclerosis-associated and experimental mutations in metal-binding residues and free cysteines lacks toxicity in vivo. Journal of Neurochemistry. 121: 475-85. PMID 22332887 DOI: 10.1111/j.1471-4159.2012.07690.x  1
2011 Prudencio M, Borchelt DR. Superoxide dismutase 1 encoding mutations linked to ALS adopts a spectrum of misfolded states. Molecular Neurodegeneration. 6: 77. PMID 22094223 DOI: 10.1186/1750-1326-6-77  1
2010 Prudencio M, Durazo A, Whitelegge JP, Borchelt DR. An examination of wild-type SOD1 in modulating the toxicity and aggregation of ALS-associated mutant SOD1. Human Molecular Genetics. 19: 4774-89. PMID 20871097 DOI: 10.1093/hmg/ddq408  1
2010 Winkler DD, Prudencio M, Karch C, Borchelt DR, Hart PJ. Copper-Zinc Superoxide Dismutase, Its Copper Chaperone, and Familial Amyotrophic Lateral Sclerosis Protein Misfolding Diseases: Current and Emerging Principles and Therapies. 381-401. DOI: 10.1002/9780470572702.ch17  1
2009 Seetharaman SV, Prudencio M, Karch C, Holloway SP, Borchelt DR, Hart PJ. Immature copper-zinc superoxide dismutase and familial amyotrophic lateral sclerosis. Experimental Biology and Medicine (Maywood, N.J.). 234: 1140-54. PMID 19596823 DOI: 10.3181/0903-MR-104  1
2009 Prudencio M, Hart PJ, Borchelt DR, Andersen PM. Variation in aggregation propensities among ALS-associated variants of SOD1: correlation to human disease. Human Molecular Genetics. 18: 3217-26. PMID 19483195 DOI: 10.1093/hmg/ddp260  1
2009 Karch CM, Prudencio M, Winkler DD, Hart PJ, Borchelt DR. Role of mutant SOD1 disulfide oxidation and aggregation in the pathogenesis of familial ALS. Proceedings of the National Academy of Sciences of the United States of America. 106: 7774-9. PMID 19416874 DOI: 10.1073/pnas.0902505106  1
2009 Prudencio M, Durazo A, Whitelegge JP, Borchelt DR. Modulation of mutant superoxide dismutase 1 aggregation by co-expression of wild-type enzyme. Journal of Neurochemistry. 108: 1009-18. PMID 19077113 DOI: 10.1111/j.1471-4159.2008.05839.x  1
2006 Hofer T, Seo AY, Prudencio M, Leeuwenburgh C. A method to determine RNA and DNA oxidation simultaneously by HPLC-ECD: greater RNA than DNA oxidation in rat liver after doxorubicin administration. Biological Chemistry. 387: 103-11. PMID 16497170 DOI: 10.1515/BC.2006.014  1
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