Thomas Jefferson University

Piera Pasinelli

TitleProfessor
InstitutionThomas Jefferson University
DepartmentCenter For ALS
Address900 Walnut Street
Philadelphia PA 19107
Phone215-955-8394
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    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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    PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. Nelson AT, Cicardi ME, Markandaiah SS, Han JY, Philp NJ, Welebob E, Haeusler AR, Pasinelli P, Manfredi G, Kawamata H, Trotti D. Glucose hypometabolism prompts RAN translation and exacerbates C9orf72-related ALS/FTD phenotypes. EMBO Rep. 2024 May; 25(5):2479-2510. PMID: 38684907.
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    2. Urban MW, Charsar BA, Heinsinger NM, Markandaiah SS, Sprimont L, Zhou W, Brown EV, Henderson NT, Thomas SJ, Ghosh B, Cain RE, Trotti D, Pasinelli P, Wright MC, Dalva MB, Lepore AC. EphrinB2 knockdown in cervical spinal cord preserves diaphragm innervation in a mutant SOD1 mouse model of ALS. Elife. 2024 Jan 15; 12. PMID: 38224498.
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    3. Pasinelli P, Meyer K, Ferraiuolo L, Culibrk RA, Sattler R. Editorial: The role of glial cells in neurodegeneration. Front Mol Med. 2023; 3:1337286. PMID: 39086672.
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    4. Urban MW, Charsar BA, Heinsinger NM, Markandaiah SS, Sprimont L, Zhou W, Henderson NT, Ghosh B, Cain RE, Trotti D, Pasinelli P, Wright MC, Dalva MB, Lepore AC. EphrinB2 knockdown in spinal cord astrocytes preserves diaphragm innervation in a mutant SOD1 mouse model of ALS. bioRxiv. 2023 May 10. PMID: 37215009.
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    5. Jensen BK, McAvoy KJ, Heinsinger NM, Lepore AC, Ilieva H, Haeusler AR, Trotti D, Pasinelli P. Targeting TNFa produced by astrocytes expressing amyotrophic lateral sclerosis-linked mutant fused in sarcoma prevents neurodegeneration and motor dysfunction in mice. Glia. 2022 Jul; 70(7):1426-1449. PMID: 35474517.
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    6. Verdone BM, Cicardi ME, Wen X, Sriramoji S, Russell K, Markandaiah SS, Jensen BK, Krishnamurthy K, Haeusler AR, Pasinelli P, Trotti D. A mouse model with widespread expression of the C9orf72-linked glycine-arginine dipeptide displays non-lethal ALS/FTD-like phenotypes. Sci Rep. 2022 04 04; 12(1):5644. PMID: 35379876.
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    7. Krishnamurthy K, Trotti D, Pasinelli P, Jensen B. Real-Time Fluorescent Measurement of Synaptic Functions in Models of Amyotrophic Lateral Sclerosis. J Vis Exp. 2021 07 16; (173). PMID: 34338673.
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    8. Krishnamurthy K, Pasinelli P. Synaptic dysfunction in amyotrophic lateral sclerosis/frontotemporal dementia: Therapeutic strategies and novel biomarkers. J Neurosci Res. 2021 Jun; 99(6):1499-1503. PMID: 33729613.
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    9. Jensen BK, Schuldi MH, McAvoy K, Russell KA, Boehringer A, Curran BM, Krishnamurthy K, Wen X, Westergard T, Ma L, Haeusler AR, Edbauer D, Pasinelli P, Trotti D. Synaptic dysfunction induced by glycine-alanine dipeptides in C9orf72-ALS/FTD is rescued by SV2 replenishment. EMBO Mol Med. 2020 Apr 29; e10722. PMID: 32347002.
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    10. Casci I, Krishnamurthy K, Kour S, Tripathy V, Ramesh N, Anderson EN, Marrone L, Grant RA, Oliver S, Gochenaur L, Patel K, Sterneckert J, Gleixner AM, Donnelly CJ, Ruepp MD, Sini AM, Zuccaro E, Pennuto M, Pasinelli P, Pandey UB. Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization. Nat Commun. 2019 Dec 06; 10(1):5583. PMID: 31811140.
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    11. Charsar BA, Brinton MA, Locke K, Chen AY, Ghosh B, Urban MW, Komaravolu S, Krishnamurthy K, Smit R, Pasinelli P, Wright MC, Smith GM, Lepore AC. AAV2-BDNF promotes respiratory axon plasticity and recovery of diaphragm function following spinal cord injury. FASEB J. 2019 Oct 02; fj201901730R. PMID: 31577916.
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    12. Mohamed LA, Markandaiah SS, Bonanno S, Pasinelli P, Trotti D. Excess glutamate secreted from astrocytes drives upregulation of P-glycoprotein in endothelial cells in amyotrophic lateral sclerosis. Exp Neurol. 2019 Jun; 316:27-38. PMID: 30974102.
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    13. Westergard T, McAvoy K, Russell K, Wen X, Pang Y, Morris B, Pasinelli P, Trotti D, Haeusler A. Repeat-associated non-AUG translation in C9orf72-ALS/FTD is driven by neuronal excitation and stress. EMBO Mol Med. 2019 Feb; 11(2). PMID: 30617154.
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    14. Kia A, McAvoy K, Krishnamurthy K, Trotti D, Pasinelli P. Astrocytes expressing ALS-linked mutant FUS induce motor neuron death through release of tumor necrosis factor-alpha. Glia. 2018 May; 66(5):1016-1033. PMID: 29380416.
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    15. Mohamed LA, Markandaiah S, Bonanno S, Pasinelli P, Trotti D. Blood-Brain Barrier Driven Pharmacoresistance in Amyotrophic Lateral Sclerosis and Challenges for Effective Drug Therapies. AAPS J. 2017 Aug 04. PMID: 28779378.
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    16. Rosenblum LT, Shamamandri-Markandaiah S, Ghosh B, Foran E, Lepore AC, Pasinelli P, Trotti D. Mutation of the caspase-3 cleavage site in the astroglial glutamate transporter EAAT2 delays disease progression and extends lifespan in the SOD1-G93A mouse model of ALS. Exp Neurol. 2017 Jun; 292:145-153. PMID: 28342750.
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    17. Westergard T, Jensen BK, Wen X, Cai J, Kropf E, Iacovitti L, Pasinelli P, Trotti D. Cell-to-Cell Transmission of Dipeptide Repeat Proteins Linked to C9orf72-ALS/FTD. Cell Rep. 2016 Oct 11; 17(3):645-652. PMID: 27732842.
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    18. Wen X, Westergard T, Pasinelli P, Trotti D. Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene. Neurosci Lett. 2017 Jan 01; 636:16-26. PMID: 27619540.
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    19. Qosa H, Lichter J, Sarlo M, Markandaiah SS, McAvoy K, Richard JP, Jablonski MR, Maragakis NJ, Pasinelli P, Trotti D. Astrocytes drive upregulation of the multidrug resistance transporter ABCB1 (P-Glycoprotein) in endothelial cells of the blood-brain barrier in mutant superoxide dismutase 1-linked amyotrophic lateral sclerosis. Glia. 2016 Aug; 64(8):1298-313. PMID: 27158936.
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    20. Daigle JG, Krishnamurthy K, Ramesh N, Casci I, Monaghan J, McAvoy K, Godfrey EW, Daniel DC, Johnson EM, Monahan Z, Shewmaker F, Pasinelli P, Pandey UB. Pur-alpha regulates cytoplasmic stress granule dynamics and ameliorates FUS toxicity. Acta Neuropathol. 2016 Apr; 131(4):605-20. PMID: 26728149.
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    21. Qosa H, Miller DS, Pasinelli P, Trotti D. Regulation of ABC efflux transporters at blood-brain barrier in health and neurological disorders. Brain Res. 2015 Dec 2; 1628(Pt B):298-316. PMID: 26187753.
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    22. Wen X, Tan W, Westergard T, Krishnamurthy K, Markandaiah SS, Shi Y, Lin S, Shneider NA, Monaghan J, Pandey UB, Pasinelli P, Ichida JK, Trotti D. Antisense Proline-Arginine RAN Dipeptides Linked to C9ORF72-ALS/FTD Form Toxic Nuclear Aggregates that Initiate In Vitro and In Vivo Neuronal Death. Neuron. 2014 Dec 17; 84(6):1213-25. PMID: 25521377.
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    23. Jablonski MR, Markandaiah SS, Jacob D, Meng NJ, Li K, Gennaro V, Lepore AC, Trotti D, Pasinelli P. Inhibiting drug efflux transporters improves efficacy of ALS therapeutics. Ann Clin Transl Neurol. 2014 Dec; 1(12):996-1005. PMID: 25574474.
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    24. Jablonski M, Miller DS, Pasinelli P, Trotti D. ABC transporter-driven pharmacoresistance in Amyotrophic Lateral Sclerosis. Brain Res. 2015 May 14; 1607:1-14. PMID: 25175835.
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    25. Tan W, Pasinelli P, Trotti D. Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis. Biochim Biophys Acta. 2014 Aug; 1842(8):1295-301. PMID: 24568860.
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    26. Shahidullah M, Le Marchand SJ, Fei H, Zhang J, Pandey UB, Dalva MB, Pasinelli P, Levitan IB. Defects in Synapse Structure and Function Precede Motor Neuron Degeneration in Drosophila Models of FUS-Related ALS. J Neurosci. 2013 Dec 11; 33(50):19590-8. PMID: 24336723.
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    27. Tan W, Naniche N, Bogush A, Pedrini S, Trotti D, Pasinelli P. Small Peptides against the Mutant SOD1/Bcl-2 Toxic Mitochondrial Complex Restore Mitochondrial Function and Cell Viability in Mutant SOD1-Mediated ALS. J Neurosci. 2013 Jul 10; 33(28):11588-98. PMID: 23843527.
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    28. Jablonski MR, Jacob DA, Campos C, Miller DS, Maragakis NJ, Pasinelli P, Trotti D. Selective increase of two ABC drug efflux transporters at the blood-spinal cord barrier suggests induced pharmacoresistance in ALS. Neurobiol Dis. 2012 Aug; 47(2):194-200. PMID: 22521463.
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    29. Guareschi S, Cova E, Cereda C, Ceroni M, Donetti E, Bosco DA, Trotti D, Pasinelli P. An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1. Proc Natl Acad Sci U S A. 2012 Mar 27; 109(13):5074-9. PMID: 22416121.
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    30. Foran E, Bogush A, Goffredo M, Roncaglia P, Gustincich S, Pasinelli P, Trotti D. Motor neuron impairment mediated by a sumoylated fragment of the glial glutamate transporter EAAT2. Glia. 2011 Nov; 59(11):1719-31. PMID: 21769946.
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    31. Naniche N, Sau D, Pasinelli P. In vivo and in vitro determination of cell death markers in neurons. Methods Mol Biol. 2011; 793:9-21. PMID: 21913091.
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    32. Bosco DA, Morfini G, Karabacak NM, Song Y, Gros-Louis F, Pasinelli P, Goolsby H, Fontaine BA, Lemay N, McKenna-Yasek D, Frosch MP, Agar JN, Julien JP, Brady ST, Brown RH. Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS. Nat Neurosci. 2010 Nov; 13(11):1396-403. PMID: 20953194.
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    33. Fieni F, Parkar A, Misgeld T, Kerschensteiner M, Lichtman JW, Pasinelli P, Trotti D. Voltage-dependent inwardly rectifying potassium conductance in the outer membrane of neuronal mitochondria. J Biol Chem. 2010 Aug 27; 285(35):27411-7. PMID: 20551319.
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    34. Pedrini S, Sau D, Guareschi S, Bogush M, Brown RH, Naniche N, Kia A, Trotti D, Pasinelli P. ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2. Hum Mol Genet. 2010 Aug 1; 19(15):2974-86. PMID: 20460269.
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    35. Boston-Howes W, Williams EO, Bogush A, Scolere M, Pasinelli P, Trotti D. Nordihydroguaiaretic acid increases glutamate uptake in vitro and in vivo: therapeutic implications for amyotrophic lateral sclerosis. Exp Neurol. 2008 Sep; 213(1):229-37. PMID: 18625223.
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    36. Gibb SL, Boston-Howes W, Lavina ZS, Gustincich S, Brown RH, Pasinelli P, Trotti D. A caspase-3-cleaved fragment of the glial glutamate transporter EAAT2 is sumoylated and targeted to promyelocytic leukemia nuclear bodies in mutant SOD1-linked amyotrophic lateral sclerosis. J Biol Chem. 2007 Nov 2; 282(44):32480-90. PMID: 17823119.
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    37. Pasinelli P, Brown RH. Molecular biology of amyotrophic lateral sclerosis: insights from genetics. Nat Rev Neurosci. 2006 Sep; 7(9):710-23. PMID: 16924260.
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    38. Boston-Howes W, Gibb SL, Williams EO, Pasinelli P, Brown RH, Trotti D. Caspase-3 cleaves and inactivates the glutamate transporter EAAT2. J Biol Chem. 2006 May 19; 281(20):14076-84. PMID: 16567804.
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    39. Broom WJ, Ay I, Pasinelli P, Brown RH. Inhibition of SOD1 expression by mitomycin C is a non-specific consequence of cellular toxicity. Neurosci Lett. 2006 Jan 30; 393(2-3):184-8. PMID: 16242241.
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    40. Pasinelli P, Belford ME, Lennon N, Bacskai BJ, Hyman BT, Trotti D, Brown RH. Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria. Neuron. 2004 Jul 8; 43(1):19-30. PMID: 15233914.
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    41. Zhu S, Li M, Figueroa BE, Liu A, Stavrovskaya IG, Pasinelli P, Beal MF, Brown RH, Kristal BS, Ferrante RJ, Friedlander RM. Prophylactic creatine administration mediates neuroprotection in cerebral ischemia in mice. J Neurosci. 2004 Jun 30; 24(26):5909-12. PMID: 15229238.
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    42. Maxwell MM, Pasinelli P, Kazantsev AG, Brown RH. RNA interference-mediated silencing of mutant superoxide dismutase rescues cyclosporin A-induced death in cultured neuroblastoma cells. Proc Natl Acad Sci U S A. 2004 Mar 2; 101(9):3178-83. PMID: 14981234.
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    43. Dangond F, Hwang D, Camelo S, Pasinelli P, Frosch MP, Stephanopoulos G, Stephanopoulos G, Brown RH, Gullans SR. Molecular signature of late-stage human ALS revealed by expression profiling of postmortem spinal cord gray matter. Physiol Genomics. 2004 Jan 15; 16(2):229-39. PMID: 14645737.
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