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Neil Aronin to Huntington Disease

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This is a "connection" page, showing publications Neil Aronin has written about Huntington Disease.
Neil Aronin
Connection Strength
10.411
Huntington Disease
  1. Aronin N. Perspective: Tominersen Testing Finds a Way Forward. J Huntingtons Dis. 2022; 11(2):119-120.
    View in: PubMed
    Score: 0.677
  2. Pfister EL, Aronin N. Huntington's Disease: Les Jeux Sont Faits? Trends Mol Med. 2020 10; 26(10):889-890.
    View in: PubMed
    Score: 0.617
  3. Pfister EL, DiNardo N, Mondo E, Borel F, Conroy F, Fraser C, Gernoux G, Han X, Hu D, Johnson E, Kennington L, Liu P, Reid SJ, Sapp E, Vodicka P, Kuchel T, Morton AJ, Howland D, Moser R, Sena-Esteves M, Gao G, Mueller C, DiFiglia M, Aronin N. Artificial miRNAs Reduce Human Mutant Huntingtin Throughout the Striatum in a Transgenic Sheep Model of Huntington's Disease. Hum Gene Ther. 2018 06; 29(6):663-673.
    View in: PubMed
    Score: 0.518
  4. Romo L, Mohn ES, Aronin N. A Fresh Look at Huntingtin mRNA Processing in Huntington's Disease. J Huntingtons Dis. 2018; 7(2):101-108.
    View in: PubMed
    Score: 0.513
  5. Romo L, Ashar-Patel A, Pfister E, Aronin N. Alterations in mRNA 3' UTR Isoform Abundance Accompany Gene Expression Changes in Human Huntington's Disease Brains. Cell Rep. 2017 Sep 26; 20(13):3057-3070.
    View in: PubMed
    Score: 0.504
  6. Liu W, Pfister EL, Kennington LA, Chase KO, Mueller C, DiFiglia M, Aronin N. Does the Mutant CAG Expansion in Huntingtin mRNA Interfere with Exonucleolytic Cleavage of its First Exon? J Huntingtons Dis. 2016; 5(1):33-8.
    View in: PubMed
    Score: 0.446
  7. Johnson E, Chase K, McGowan S, Mondo E, Pfister E, Mick E, Friedline RH, Kim JK, Sapp E, DiFiglia M, Aronin N. Safety of Striatal Infusion of siRNA in a Transgenic Huntington's Disease Mouse Model. J Huntingtons Dis. 2015; 4(3):219-229.
    View in: PubMed
    Score: 0.417
  8. Aronin N, DiFiglia M. Huntingtin-lowering strategies in Huntington's disease: antisense oligonucleotides, small RNAs, and gene editing. Mov Disord. 2014 Sep 15; 29(11):1455-61.
    View in: PubMed
    Score: 0.407
  9. Liu W, Chaurette J, Pfister EL, Kennington LA, Chase KO, Bullock J, Vonsattel JP, Faull RL, Macdonald D, DiFiglia M, Zamore PD, Aronin N. Increased Steady-State Mutant Huntingtin mRNA in Huntington's Disease Brain. J Huntingtons Dis. 2013; 2(4):491-500.
    View in: PubMed
    Score: 0.363
  10. van der Bom IM, Moser RP, Gao G, Mondo E, O'Connell D, Gounis MJ, McGowan S, Chaurette J, Bishop N, Sena-Esteves MS, Mueller C, Aronin N. Finding the striatum in sheep: use of a multi-modal guided approach for convection enhanced delivery. J Huntingtons Dis. 2013; 2(1):41-5.
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    Score: 0.363
  11. Aronin N, Moore M. Hunting down huntingtin. N Engl J Med. 2012 11 01; 367(18):1753-4.
    View in: PubMed
    Score: 0.359
  12. Sah DW, Aronin N. Oligonucleotide therapeutic approaches for Huntington disease. J Clin Invest. 2011 Feb; 121(2):500-7.
    View in: PubMed
    Score: 0.318
  13. Aronin N. Expanded CAG repeats in the crosshairs. Nat Biotechnol. 2009 May; 27(5):451-2.
    View in: PubMed
    Score: 0.281
  14. Pfister EL, Kennington L, Straubhaar J, Wagh S, Liu W, DiFiglia M, Landwehrmeyer B, Vonsattel JP, Zamore PD, Aronin N. Five siRNAs targeting three SNPs may provide therapy for three-quarters of Huntington's disease patients. Curr Biol. 2009 May 12; 19(9):774-8.
    View in: PubMed
    Score: 0.280
  15. Liu W, Kennington LA, Rosas HD, Hersch S, Cha JH, Zamore PD, Aronin N. Linking SNPs to CAG repeat length in Huntington's disease patients. Nat Methods. 2008 Nov; 5(11):951-3.
    View in: PubMed
    Score: 0.271
  16. Allen S, O'Reilly D, Miller R, Sapp E, Summers A, Paquette J, Echeverria Moreno D, Bramato B, McHugh N, Yamada K, Aronin N, DiFiglia M, Khvorova A. mRNA Nuclear Clustering Leads to a Difference in Mutant Huntingtin mRNA and Protein Silencing by siRNAs In Vivo. Nucleic Acid Ther. 2024 08; 34(4):164-172.
    View in: PubMed
    Score: 0.202
  17. O'Reilly D, Belgrad J, Ferguson C, Summers A, Sapp E, McHugh C, Mathews E, Boudi A, Buchwald J, Ly S, Moreno D, Furgal R, Luu E, Kennedy Z, Hariharan V, Monopoli K, Yang XW, Carroll J, DiFiglia M, Aronin N, Khvorova A. Di-valent siRNA-mediated silencing of MSH3 blocks somatic repeat expansion in mouse models of Huntington's disease. Mol Ther. 2023 06 07; 31(6):1661-1674.
    View in: PubMed
    Score: 0.186
  18. Conroy F, Miller R, Alterman JF, Hassler MR, Echeverria D, Godinho BMDC, Knox EG, Sapp E, Sousa J, Yamada K, Mahmood F, Boudi A, Kegel-Gleason K, DiFiglia M, Aronin N, Khvorova A, Pfister EL. Chemical engineering of therapeutic siRNAs for allele-specific gene silencing in Huntington's disease models. Nat Commun. 2022 10 03; 13(1):5802.
    View in: PubMed
    Score: 0.178
  19. Peters?n A, Chase K, Puschban Z, DiFiglia M, Brundin P, Aronin N. Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease. Exp Neurol. 2002 May; 175(1):297-300.
    View in: PubMed
    Score: 0.173
  20. Oikemus SR, Pfister EL, Sapp E, Chase KO, Kennington LA, Hudgens E, Miller R, Zhu LJ, Chaudhary A, Mick EO, Sena-Esteves M, Wolfe SA, DiFiglia M, Aronin N, Brodsky MH. Allele-Specific Knockdown of Mutant Huntingtin Protein via Editing at Coding Region Single Nucleotide Polymorphism Heterozygosities. Hum Gene Ther. 2022 01; 33(1-2):25-36.
    View in: PubMed
    Score: 0.169
  21. Yamada K, Hildebrand S, Davis SM, Miller R, Conroy F, Sapp E, Caiazzi J, Alterman JF, Roux L, Echeverria D, Hassler MR, Pfister EL, DiFiglia M, Aronin N, Khvorova A. Structurally constrained phosphonate internucleotide linkage impacts oligonucleotide-enzyme interaction, and modulates siRNA activity and allele specificity. Nucleic Acids Res. 2021 12 02; 49(21):12069-12088.
    View in: PubMed
    Score: 0.168
  22. Laforet GA, Sapp E, Chase K, McIntyre C, Boyce FM, Campbell M, Cadigan BA, Warzecki L, Tagle DA, Reddy PH, Cepeda C, Calvert CR, Jokel ES, Klapstein GJ, Ariano MA, Levine MS, DiFiglia M, Aronin N. Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease. J Neurosci. 2001 Dec 01; 21(23):9112-23.
    View in: PubMed
    Score: 0.168
  23. Howland D, Ellederova Z, Aronin N, Fernau D, Gallagher J, Taylor A, Hennebold J, Weiss AR, Gray-Edwards H, McBride J. Large Animal Models of Huntington's Disease: What We Have Learned and Where We Need to Go Next. J Huntingtons Dis. 2020; 9(3):201-216.
    View in: PubMed
    Score: 0.147
  24. Aronin N, Kim M, Laforet G, DiFiglia M. Are there multiple pathways in the pathogenesis of Huntington's disease? Philos Trans R Soc Lond B Biol Sci. 1999 Jun 29; 354(1386):995-1003.
    View in: PubMed
    Score: 0.142
  25. Tousley A, Iuliano M, Weisman E, Sapp E, Zhang N, Vodicka P, Alexander J, Aviolat H, Gatune L, Reeves P, Li X, Khvorova A, Ellerby LM, Aronin N, DiFiglia M, Kegel-Gleason KB. Rac1 Activity Is Modulated by Huntingtin and Dysregulated in Models of Huntington's Disease. J Huntingtons Dis. 2019; 8(1):53-69.
    View in: PubMed
    Score: 0.137
  26. Didiot MC, Ferguson CM, Ly S, Coles AH, Smith AO, Bicknell AA, Hall LM, Sapp E, Echeverria D, Pai AA, DiFiglia M, Moore MJ, Hayward LJ, Aronin N, Khvorova A. Nuclear Localization of Huntingtin mRNA Is Specific to Cells of Neuronal Origin. Cell Rep. 2018 09 04; 24(10):2553-2560.e5.
    View in: PubMed
    Score: 0.134
  27. DiFiglia M, Sapp E, Chase KO, Davies SW, Bates GP, Vonsattel JP, Aronin N. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science. 1997 Sep 26; 277(5334):1990-3.
    View in: PubMed
    Score: 0.126
  28. Miller JRC, Pfister EL, Liu W, Andre R, Tr?ger U, Kennington LA, Lo K, Dijkstra S, Macdonald D, Ostroff G, Aronin N, Tabrizi SJ. Allele-Selective Suppression of Mutant Huntingtin in Primary Human Blood Cells. Sci Rep. 2017 04 24; 7:46740.
    View in: PubMed
    Score: 0.122
  29. Vodicka P, Chase K, Iuliano M, Tousley A, Valentine DT, Sapp E, Kegel-Gleason KB, Sena-Esteves M, Aronin N, DiFiglia M. Autophagy Activation by Transcription Factor EB (TFEB) in Striatum of HDQ175/Q7 Mice. J Huntingtons Dis. 2016 10 01; 5(3):249-260.
    View in: PubMed
    Score: 0.118
  30. Keeler AM, Sapp E, Chase K, Sottosanti E, Danielson E, Pfister E, Stoica L, DiFiglia M, Aronin N, Sena-Esteves M. Cellular Analysis of Silencing the?Huntington's Disease Gene Using AAV9?Mediated Delivery of Artificial Micro?RNA into the Striatum of?Q140/Q140?Mice. J Huntingtons Dis. 2016 10 01; 5(3):239-248.
    View in: PubMed
    Score: 0.118
  31. Vodicka P, Chase K, Iuliano M, Valentine DT, Sapp E, Lu B, Kegel-Gleason KB, Sena-Esteves M, Aronin N, DiFiglia M. Effects of Exogenous NUB1 Expression in the Striatum of HDQ175/Q7 Mice. J Huntingtons Dis. 2016 06 13; 5(2):163-74.
    View in: PubMed
    Score: 0.115
  32. Aronin N, Chase K, Young C, Sapp E, Schwarz C, Matta N, Kornreich R, Landwehrmeyer B, Bird E, Beal MF, et al. CAG expansion affects the expression of mutant Huntingtin in the Huntington's disease brain. Neuron. 1995 Nov; 15(5):1193-201.
    View in: PubMed
    Score: 0.110
  33. Vodicka P, Mo S, Tousley A, Green KM, Sapp E, Iuliano M, Sadri-Vakili G, Shaffer SA, Aronin N, DiFiglia M, Kegel-Gleason KB. Mass Spectrometry Analysis of Wild-Type and Knock-in Q140/Q140 Huntington's Disease Mouse Brains Reveals Changes in Glycerophospholipids Including Alterations in Phosphatidic Acid and Lyso-Phosphatidic Acid. J Huntingtons Dis. 2015; 4(2):187-201.
    View in: PubMed
    Score: 0.104
  34. Tr?ger U, Andre R, Lahiri N, Magnusson-Lind A, Weiss A, Grueninger S, McKinnon C, Sirinathsinghji E, Kahlon S, Pfister EL, Moser R, Hummerich H, Antoniou M, Bates GP, Luthi-Carter R, Lowdell MW, Bj?rkqvist M, Ostroff GR, Aronin N, Tabrizi SJ. HTT-lowering reverses Huntington's disease immune dysfunction caused by NF?B pathway dysregulation. Brain. 2014 Mar; 137(Pt 3):819-33.
    View in: PubMed
    Score: 0.098
  35. Vodicka P, Lim J, Williams DT, Kegel KB, Chase K, Park H, Marchionini D, Wilkinson S, Mead T, Birch H, Yates D, Lyons K, Dominguez C, Beconi M, Yue Z, Aronin N, DiFiglia M. Assessment of chloroquine treatment for modulating autophagy flux in brain of WT and HD mice. J Huntingtons Dis. 2014; 3(2):159-74.
    View in: PubMed
    Score: 0.097
  36. Valencia A, Sapp E, Kimm JS, McClory H, Ansong KA, Yohrling G, Kwak S, Kegel KB, Green KM, Shaffer SA, Aronin N, DiFiglia M. Striatal synaptosomes from Hdh140Q/140Q knock-in mice have altered protein levels, novel sites of methionine oxidation, and excess glutamate release after stimulation. J Huntingtons Dis. 2013; 2(4):459-75.
    View in: PubMed
    Score: 0.091
  37. Sapp E, Valencia A, Li X, Aronin N, Kegel KB, Vonsattel JP, Young AB, Wexler N, DiFiglia M. Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer. J Biol Chem. 2012 Apr 13; 287(16):13487-99.
    View in: PubMed
    Score: 0.086
  38. Li X, Valencia A, Sapp E, Masso N, Alexander J, Reeves P, Kegel KB, Aronin N, Difiglia M. Aberrant Rab11-dependent trafficking of the neuronal glutamate transporter EAAC1 causes oxidative stress and cell death in Huntington's disease. J Neurosci. 2010 Mar 31; 30(13):4552-61.
    View in: PubMed
    Score: 0.075
  39. Valencia A, Reeves PB, Sapp E, Li X, Alexander J, Kegel KB, Chase K, Aronin N, DiFiglia M. Mutant huntingtin and glycogen synthase kinase 3-beta accumulate in neuronal lipid rafts of a presymptomatic knock-in mouse model of Huntington's disease. J Neurosci Res. 2010 Jan; 88(1):179-90.
    View in: PubMed
    Score: 0.074
  40. Li X, Sapp E, Chase K, Comer-Tierney LA, Masso N, Alexander J, Reeves P, Kegel KB, Valencia A, Esteves M, Aronin N, Difiglia M. Disruption of Rab11 activity in a knock-in mouse model of Huntington's disease. Neurobiol Dis. 2009 Nov; 36(2):374-83.
    View in: PubMed
    Score: 0.072
  41. Kegel KB, Sapp E, Alexander J, Valencia A, Reeves P, Li X, Masso N, Sobin L, Aronin N, DiFiglia M. Polyglutamine expansion in huntingtin alters its interaction with phospholipids. J Neurochem. 2009 Sep; 110(5):1585-97.
    View in: PubMed
    Score: 0.071
  42. DiFiglia M, Sena-Esteves M, Chase K, Sapp E, Pfister E, Sass M, Yoder J, Reeves P, Pandey RK, Rajeev KG, Manoharan M, Sah DW, Zamore PD, Aronin N. Therapeutic silencing of mutant huntingtin with siRNA attenuates striatal and cortical neuropathology and behavioral deficits. Proc Natl Acad Sci U S A. 2007 Oct 23; 104(43):17204-9.
    View in: PubMed
    Score: 0.063
  43. Aronin N. Target selectivity in mRNA silencing. Gene Ther. 2006 Mar; 13(6):509-16.
    View in: PubMed
    Score: 0.056
  44. Kim YJ, Sapp E, Cuiffo BG, Sobin L, Yoder J, Kegel KB, Qin ZH, Detloff P, Aronin N, DiFiglia M. Lysosomal proteases are involved in generation of N-terminal huntingtin fragments. Neurobiol Dis. 2006 May; 22(2):346-56.
    View in: PubMed
    Score: 0.056
  45. Ariano MA, Cepeda C, Calvert CR, Flores-Hern?ndez J, Hern?ndez-Echeagaray E, Klapstein GJ, Chandler SH, Aronin N, DiFiglia M, Levine MS. Striatal potassium channel dysfunction in Huntington's disease transgenic mice. J Neurophysiol. 2005 May; 93(5):2565-74.
    View in: PubMed
    Score: 0.052
  46. Belgrad J, Tang Q, Hildebrand S, Summers A, Sapp E, Echeverria D, O'Reilly D, Luu E, Bramato B, Allen S, Cooper D, Alterman J, Yamada K, Aronin N, DiFiglia M, Khvorova A. A programmable dual-targeting siRNA scaffold supports potent two-gene modulation in the central nervous system. Nucleic Acids Res. 2024 Jun 24; 52(11):6099-6113.
    View in: PubMed
    Score: 0.050
  47. Qin ZH, Wang Y, Sapp E, Cuiffo B, Wanker E, Hayden MR, Kegel KB, Aronin N, DiFiglia M. Huntingtin bodies sequester vesicle-associated proteins by a polyproline-dependent interaction. J Neurosci. 2004 Jan 07; 24(1):269-81.
    View in: PubMed
    Score: 0.049
  48. Qin ZH, Wang Y, Kegel KB, Kazantsev A, Apostol BL, Thompson LM, Yoder J, Aronin N, DiFiglia M. Autophagy regulates the processing of amino terminal huntingtin fragments. Hum Mol Genet. 2003 Dec 15; 12(24):3231-44.
    View in: PubMed
    Score: 0.048
  49. Aronin N, Cooper PE, Lorenz LJ, Bird ED, Sagar SM, Leeman SE, Martin JB. Somatostatin is increased in the basal ganglia in Huntington disease. Ann Neurol. 1983 May; 13(5):519-26.
    View in: PubMed
    Score: 0.046
  50. Chan EY, Luthi-Carter R, Strand A, Solano SM, Hanson SA, DeJohn MM, Kooperberg C, Chase KO, DiFiglia M, Young AB, Leavitt BR, Cha JH, Aronin N, Hayden MR, Olson JM. Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease. Hum Mol Genet. 2002 Aug 15; 11(17):1939-51.
    View in: PubMed
    Score: 0.044
  51. Ariano MA, Aronin N, Difiglia M, Tagle DA, Sibley DR, Leavitt BR, Hayden MR, Levine MS. Striatal neurochemical changes in transgenic models of Huntington's disease. J Neurosci Res. 2002 Jun 15; 68(6):716-29.
    View in: PubMed
    Score: 0.044
  52. Kim YJ, Yi Y, Sapp E, Wang Y, Cuiffo B, Kegel KB, Qin ZH, Aronin N, DiFiglia M. Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis. Proc Natl Acad Sci U S A. 2001 Oct 23; 98(22):12784-9.
    View in: PubMed
    Score: 0.042
  53. Sapp E, Kegel KB, Aronin N, Hashikawa T, Uchiyama Y, Tohyama K, Bhide PG, Vonsattel JP, DiFiglia M. Early and progressive accumulation of reactive microglia in the Huntington disease brain. J Neuropathol Exp Neurol. 2001 Feb; 60(2):161-72.
    View in: PubMed
    Score: 0.040
  54. Kegel KB, Kim M, Sapp E, McIntyre C, Casta?o JG, Aronin N, DiFiglia M. Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy. J Neurosci. 2000 Oct 01; 20(19):7268-78.
    View in: PubMed
    Score: 0.039
  55. Tousley A, Iuliano M, Weisman E, Sapp E, Richardson H, Vodicka P, Alexander J, Aronin N, DiFiglia M, Kegel-Gleason KB. Huntingtin associates with the actin cytoskeleton and a-actinin isoforms to influence stimulus dependent morphology changes. PLoS One. 2019; 14(2):e0212337.
    View in: PubMed
    Score: 0.035
  56. Sapp E, Penney J, Young A, Aronin N, Vonsattel JP, DiFiglia M. Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease. J Neuropathol Exp Neurol. 1999 Feb; 58(2):165-73.
    View in: PubMed
    Score: 0.035
  57. Davies SW, Beardsall K, Turmaine M, DiFiglia M, Aronin N, Bates GP. Are neuronal intranuclear inclusions the common neuropathology of triplet-repeat disorders with polyglutamine-repeat expansions? Lancet. 1998 Jan 10; 351(9096):131-3.
    View in: PubMed
    Score: 0.032
  58. Sapp E, Schwarz C, Chase K, Bhide PG, Young AB, Penney J, Vonsattel JP, Aronin N, DiFiglia M. Huntingtin localization in brains of normal and Huntington's disease patients. Ann Neurol. 1997 Oct; 42(4):604-12.
    View in: PubMed
    Score: 0.032
  59. Kegel KB, Meloni AR, Yi Y, Kim YJ, Doyle E, Cuiffo BG, Sapp E, Wang Y, Qin ZH, Chen JD, Nevins JR, Aronin N, DiFiglia M. Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J Biol Chem. 2002 Mar 01; 277(9):7466-76.
    View in: PubMed
    Score: 0.011
  60. Martin EJ, Kim M, Velier J, Sapp E, Lee HS, Laforet G, Won L, Chase K, Bhide PG, Heller A, Aronin N, Difiglia M. Analysis of Huntingtin-associated protein 1 in mouse brain and immortalized striatal neurons. J Comp Neurol. 1999 Jan 25; 403(4):421-30.
    View in: PubMed
    Score: 0.009
  61. Kim M, Velier J, Chase K, Laforet G, Kalchman MA, Hayden MR, Won L, Heller A, Aronin N, Difiglia M. Forskolin and dopamine D1 receptor activation increase huntingtin's association with endosomes in immortalized neuronal cells of striatal origin. Neuroscience. 1999; 89(4):1159-67.
    View in: PubMed
    Score: 0.009
  62. Bhide PG, Day M, Sapp E, Schwarz C, Sheth A, Kim J, Young AB, Penney J, Golden J, Aronin N, DiFiglia M. Expression of normal and mutant huntingtin in the developing brain. J Neurosci. 1996 Sep 01; 16(17):5523-35.
    View in: PubMed
    Score: 0.007
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