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Items: 45


Repeat-associated non-AUG translation in C9orf72-ALS/FTD is driven by neuronal excitation and stress.

Westergard T, McAvoy K, Russell K, Wen X, Pang Y, Morris B, Pasinelli P, Trotti D, Haeusler A.

EMBO Mol Med. 2019 Jan 7. pii: e9423. doi: 10.15252/emmm.201809423. [Epub ahead of print]


Astrocytes expressing ALS-linked mutant FUS induce motor neuron death through release of tumor necrosis factor-alpha.

Kia A, McAvoy K, Krishnamurthy K, Trotti D, Pasinelli P.

Glia. 2018 May;66(5):1016-1033. doi: 10.1002/glia.23298. Epub 2018 Jan 30.


Blood-Brain Barrier Driven Pharmacoresistance in Amyotrophic Lateral Sclerosis and Challenges for Effective Drug Therapies.

Mohamed LA, Markandaiah S, Bonanno S, Pasinelli P, Trotti D.

AAPS J. 2017 Nov;19(6):1600-1614. doi: 10.1208/s12248-017-0120-6. Epub 2017 Aug 4. Review.


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.

Rosenblum LT, Shamamandri-Markandaiah S, Ghosh B, Foran E, Lepore AC, Pasinelli P, Trotti D.

Exp Neurol. 2017 Jun;292:145-153. doi: 10.1016/j.expneurol.2017.03.014. Epub 2017 Mar 22.


Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.

Wen X, Westergard T, Pasinelli P, Trotti D.

Neurosci Lett. 2017 Jan 1;636:16-26. doi: 10.1016/j.neulet.2016.09.007. Epub 2016 Sep 13. Review.


Cell-to-Cell Transmission of Dipeptide Repeat Proteins Linked to C9orf72-ALS/FTD.

Westergard T, Jensen BK, Wen X, Cai J, Kropf E, Iacovitti L, Pasinelli P, Trotti D.

Cell Rep. 2016 Oct 11;17(3):645-652. doi: 10.1016/j.celrep.2016.09.032.


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.

Qosa H, Lichter J, Sarlo M, Markandaiah SS, McAvoy K, Richard JP, Jablonski MR, Maragakis NJ, Pasinelli P, Trotti D.

Glia. 2016 Aug;64(8):1298-313. doi: 10.1002/glia.23003. Epub 2016 May 9.


Pur-alpha regulates cytoplasmic stress granule dynamics and ameliorates FUS toxicity.

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.

Acta Neuropathol. 2016 Apr;131(4):605-20. doi: 10.1007/s00401-015-1530-0. Epub 2016 Jan 4.


Regulation of ABC efflux transporters at blood-brain barrier in health and neurological disorders.

Qosa H, Miller DS, Pasinelli P, Trotti D.

Brain Res. 2015 Dec 2;1628(Pt B):298-316. doi: 10.1016/j.brainres.2015.07.005. Epub 2015 Jul 15. Review.


Inhibiting drug efflux transporters improves efficacy of ALS therapeutics.

Jablonski MR, Markandaiah SS, Jacob D, Meng NJ, Li K, Gennaro V, Lepore AC, Trotti D, Pasinelli P.

Ann Clin Transl Neurol. 2014 Dec;1(12):996-1005. doi: 10.1002/acn3.141. Epub 2014 Nov 21.


Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death.

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.

Neuron. 2014 Dec 17;84(6):1213-25. doi: 10.1016/j.neuron.2014.12.010.


ABC transporter-driven pharmacoresistance in Amyotrophic Lateral Sclerosis.

Jablonski M, Miller DS, Pasinelli P, Trotti D.

Brain Res. 2015 May 14;1607:1-14. doi: 10.1016/j.brainres.2014.08.060. Epub 2014 Aug 28. Review.


Sumoylation of the astroglial glutamate transporter EAAT2 governs its intracellular compartmentalization.

Foran E, Rosenblum L, Bogush A, Pasinelli P, Trotti D.

Glia. 2014 Aug;62(8):1241-53. doi: 10.1002/glia.22677. Epub 2014 Apr 21.


Role of mitochondria in mutant SOD1 linked amyotrophic lateral sclerosis.

Tan W, Pasinelli P, Trotti D.

Biochim Biophys Acta. 2014 Aug;1842(8):1295-301. doi: 10.1016/j.bbadis.2014.02.009. Epub 2014 Feb 22. Review.


Defects in synapse structure and function precede motor neuron degeneration in Drosophila models of FUS-related ALS.

Shahidullah M, Le Marchand SJ, Fei H, Zhang J, Pandey UB, Dalva MB, Pasinelli P, Levitan IB.

J Neurosci. 2013 Dec 11;33(50):19590-8. doi: 10.1523/JNEUROSCI.3396-13.2013.


Small peptides against the mutant SOD1/Bcl-2 toxic mitochondrial complex restore mitochondrial function and cell viability in mutant SOD1-mediated ALS.

Tan W, Naniche N, Bogush A, Pedrini S, Trotti D, Pasinelli P.

J Neurosci. 2013 Jul 10;33(28):11588-98. doi: 10.1523/JNEUROSCI.5385-12.2013.


Selective increase of two ABC drug efflux transporters at the blood-spinal cord barrier suggests induced pharmacoresistance in ALS.

Jablonski MR, Jacob DA, Campos C, Miller DS, Maragakis NJ, Pasinelli P, Trotti D.

Neurobiol Dis. 2012 Aug;47(2):194-200. doi: 10.1016/j.nbd.2012.03.040. Epub 2012 Apr 11.


An over-oxidized form of superoxide dismutase found in sporadic amyotrophic lateral sclerosis with bulbar onset shares a toxic mechanism with mutant SOD1.

Guareschi S, Cova E, Cereda C, Ceroni M, Donetti E, Bosco DA, Trotti D, Pasinelli P.

Proc Natl Acad Sci U S A. 2012 Mar 27;109(13):5074-9. doi: 10.1073/pnas.1115402109. Epub 2012 Mar 13.


In vivo and in vitro determination of cell death markers in neurons.

Naniche N, Sau D, Pasinelli P.

Methods Mol Biol. 2011;793:9-21. doi: 10.1007/978-1-61779-328-8_2.


Motor neuron impairment mediated by a sumoylated fragment of the glial glutamate transporter EAAT2.

Foran E, Bogush A, Goffredo M, Roncaglia P, Gustincich S, Pasinelli P, Trotti D.

Glia. 2011 Nov;59(11):1719-31. doi: 10.1002/glia.21218. Epub 2011 Jul 18.


Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.

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 Jr.

Nat Neurosci. 2010 Nov;13(11):1396-403. doi: 10.1038/nn.2660. Epub 2010 Oct 17.


Voltage-dependent inwardly rectifying potassium conductance in the outer membrane of neuronal mitochondria.

Fieni F, Parkar A, Misgeld T, Kerschensteiner M, Lichtman JW, Pasinelli P, Trotti D.

J Biol Chem. 2010 Aug 27;285(35):27411-7. doi: 10.1074/jbc.M110.131243. Epub 2010 Jun 15.


ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2.

Pedrini S, Sau D, Guareschi S, Bogush M, Brown RH Jr, Naniche N, Kia A, Trotti D, Pasinelli P.

Hum Mol Genet. 2010 Aug 1;19(15):2974-86. doi: 10.1093/hmg/ddq202. Epub 2010 May 11.


Nordihydroguaiaretic acid increases glutamate uptake in vitro and in vivo: therapeutic implications for amyotrophic lateral sclerosis.

Boston-Howes W, Williams EO, Bogush A, Scolere M, Pasinelli P, Trotti D.

Exp Neurol. 2008 Sep;213(1):229-37. doi: 10.1016/j.expneurol.2008.06.010. Epub 2008 Jun 24.


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.

Gibb SL, Boston-Howes W, Lavina ZS, Gustincich S, Brown RH Jr, Pasinelli P, Trotti D.

J Biol Chem. 2007 Nov 2;282(44):32480-90. Epub 2007 Sep 6.


The proapoptotic BCL-2 family member BIM mediates motoneuron loss in a model of amyotrophic lateral sclerosis.

Hetz C, Thielen P, Fisher J, Pasinelli P, Brown RH, Korsmeyer S, Glimcher L.

Cell Death Differ. 2007 Jul;14(7):1386-9. Epub 2007 May 18. No abstract available.


Molecular biology of amyotrophic lateral sclerosis: insights from genetics.

Pasinelli P, Brown RH.

Nat Rev Neurosci. 2006 Sep;7(9):710-23. Review.


Caspase-3 cleaves and inactivates the glutamate transporter EAAT2.

Boston-Howes W, Gibb SL, Williams EO, Pasinelli P, Brown RH Jr, Trotti D.

J Biol Chem. 2006 May 19;281(20):14076-84. Epub 2006 Mar 27.


Inhibition of SOD1 expression by mitomycin C is a non-specific consequence of cellular toxicity.

Broom WJ, Ay I, Pasinelli P, Brown RH Jr.

Neurosci Lett. 2006 Jan 30;393(2-3):184-8. Epub 2005 Oct 19.


Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria.

Pasinelli P, Belford ME, Lennon N, Bacskai BJ, Hyman BT, Trotti D, Brown RH Jr.

Neuron. 2004 Jul 8;43(1):19-30.


Prophylactic creatine administration mediates neuroprotection in cerebral ischemia in mice.

Zhu S, Li M, Figueroa BE, Liu A, Stavrovskaya IG, Pasinelli P, Beal MF, Brown RH Jr, Kristal BS, Ferrante RJ, Friedlander RM.

J Neurosci. 2004 Jun 30;24(26):5909-12.


RNA interference-mediated silencing of mutant superoxide dismutase rescues cyclosporin A-induced death in cultured neuroblastoma cells.

Maxwell MM, Pasinelli P, Kazantsev AG, Brown RH Jr.

Proc Natl Acad Sci U S A. 2004 Mar 2;101(9):3178-83. Epub 2004 Feb 23.


Molecular signature of late-stage human ALS revealed by expression profiling of postmortem spinal cord gray matter.

Dangond F, Hwang D, Camelo S, Pasinelli P, Frosch MP, Stephanopoulos G, Stephanopoulos G, Brown RH Jr, Gullans SR.

Physiol Genomics. 2004 Jan 15;16(2):229-39.


Survival in transgenic ALS mice does not vary with CNS glutathione peroxidase activity.

Cudkowicz ME, Pastusza KA, Sapp PC, Mathews RK, Leahy J, Pasinelli P, Francis JW, Jiang D, Andersen JK, Brown RH Jr.

Neurology. 2002 Sep 10;59(5):729-34.


Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: associated mutations develop motor neuron disease.

Nagai M, Aoki M, Miyoshi I, Kato M, Pasinelli P, Kasai N, Brown RH Jr, Itoyama Y.

J Neurosci. 2001 Dec 1;21(23):9246-54.


Caspase-1 and -3 are sequentially activated in motor neuron death in Cu,Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis.

Pasinelli P, Houseweart MK, Brown RH Jr, Cleveland DW.

Proc Natl Acad Sci U S A. 2000 Dec 5;97(25):13901-6.


Amyotrophic lateral sclerosis-linked glutamate transporter mutant has impaired glutamate clearance capacity.

Trotti D, Aoki M, Pasinelli P, Berger UV, Danbolt NC, Brown RH Jr, Hediger MA.

J Biol Chem. 2001 Jan 5;276(1):576-82.


Activation of pre- and postsynaptic protein kinase C during tetraethylammonium-induced long-term potentiation in the CA1 field of the hippocampus.

Ramakers GM, Pasinelli P, van Beest M, van der Slot A, Gispen WH, De Graan PN.

Neurosci Lett. 2000 May 26;286(1):53-6.


Decreased intracellular superoxide levels activate Sindbis virus-induced apoptosis.

Lin KI, Pasinelli P, Brown RH, Hardwick JM, Ratan RR.

J Biol Chem. 1999 May 7;274(19):13650-5.


Caspase-1 is activated in neural cells and tissue with amyotrophic lateral sclerosis-associated mutations in copper-zinc superoxide dismutase.

Pasinelli P, Borchelt DR, Houseweart MK, Cleveland DW, Brown RH Jr.

Proc Natl Acad Sci U S A. 1998 Dec 22;95(26):15763-8. Erratum in: Proc Natl Acad Sci U S A 1999 Mar 16;96(6):3330.


Phosphorylation of the casein kinase II domain of B-50 (GAP-43) in rat cortical growth cones.

Edgar MA, Pasinelli P, DeWit M, Anton B, Dokas LA, Pastorino L, DiLuca M, Cattabeni F, Gispen WH, De Graan PN.

J Neurochem. 1997 Nov;69(5):2206-15.


Protein kinase C in synaptic plasticity: changes in the in situ phosphorylation state of identified pre- and postsynaptic substrates.

Ramakers GM, Pasinelli P, Hens JJ, Gispen WH, De Graan PN.

Prog Neuropsychopharmacol Biol Psychiatry. 1997 Apr;21(3):455-86. Review.


Temporal differences in the phosphorylation state of pre- and postsynaptic protein kinase C substrates B-50/GAP-43 and neurogranin during long-term potentiation.

Ramakers GM, De Graan PN, Urban IJ, Kraay D, Tang T, Pasinelli P, Oestreicher AB, Gispen WH.

J Biol Chem. 1995 Jun 9;270(23):13892-8.


Antibodies to postsynaptic PKC substrate neurogranin prevent long-term potentiation in hippocampal CA1 neurons.

Fedorov NB, Pasinelli P, Oestreicher AB, DeGraan PN, Reymann KG.

Eur J Neurosci. 1995 Apr 1;7(4):819-22.


Long-term potentiation and synaptic protein phosphorylation.

Pasinelli P, Ramakers GM, Urban IJ, Hens JJ, Oestreicher AB, de Graan PN, Gispen WH.

Behav Brain Res. 1995 Jan 23;66(1-2):53-9. Review.


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