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Connection

Neil Aronin to Huntingtin Protein

This is a "connection" page, showing publications Neil Aronin has written about Huntingtin Protein.
Connection Strength

4.860
  1. 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: 0.537
  2. 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: 0.532
  3. Mondo E, Moser R, Gao G, Mueller C, Sena-Esteves M, Sapp E, Pfister E, O'Connell D, Takle K, Erger KE, Liu W, Conlon TJ, DiFiglia M, Gounis MJ, Aronin N. Selective Neuronal Uptake and Distribution of AAVrh8, AAV9, and AAVrh10 in Sheep After Intra-Striatal Administration. J Huntingtons Dis. 2018; 7(4):309-319.
    View in: PubMed
    Score: 0.532
  4. 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: 0.463
  5. Iwanowicz A, Boudi A, Seeley C, Sapp E, Miller R, Liu S, Chase K, Shing K, Batista AR, Siena-Esteves M, Aronin N, DiFiglia M, Kegel-Gleason KB. Intrastriatal Delivery of a Zinc Finger Protein Targeting the Mutant HTT Gene Allele Obviates Lipid Phenotypes in Brain and Plasma in Huntington's Disease?Mice. Hum Gene Ther. 2025 Aug; 36(15-16):1083-1094.
    View in: PubMed
    Score: 0.224
  6. 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: 0.209
  7. Alterman JF, Godinho BMDC, Hassler MR, Ferguson CM, Echeverria D, Sapp E, Haraszti RA, Coles AH, Conroy F, Miller R, Roux L, Yan P, Knox EG, Turanov AA, King RM, Gernoux G, Mueller C, Gray-Edwards HL, Moser RP, Bishop NC, Jaber SM, Gounis MJ, Sena-Esteves M, Pai AA, DiFiglia M, Aronin N, Khvorova A. A divalent siRNA chemical scaffold for potent and sustained modulation of gene expression throughout the central nervous system. Nat Biotechnol. 2019 08; 37(8):884-894.
    View in: PubMed
    Score: 0.148
  8. 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: 0.144
  9. Godinho BMDC, Henninger N, Bouley J, Alterman JF, Haraszti RA, Gilbert JW, Sapp E, Coles AH, Biscans A, Nikan M, Echeverria D, DiFiglia M, Aronin N, Khvorova A. Transvascular Delivery of Hydrophobically Modified siRNAs: Gene Silencing in the Rat Brain upon Disruption of the Blood-Brain Barrier. Mol Ther. 2018 11 07; 26(11):2580-2591.
    View in: PubMed
    Score: 0.139
  10. Haraszti RA, Miller R, Didiot MC, Biscans A, Alterman JF, Hassler MR, Roux L, Echeverria D, Sapp E, DiFiglia M, Aronin N, Khvorova A. Optimized Cholesterol-siRNA Chemistry Improves Productive Loading onto Extracellular Vesicles. Mol Ther. 2018 08 01; 26(8):1973-1982.
    View in: PubMed
    Score: 0.137
  11. 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: 0.127
  12. Didiot MC, Hall LM, Coles AH, Haraszti RA, Godinho BM, Chase K, Sapp E, Ly S, Alterman JF, Hassler MR, Echeverria D, Raj L, Morrissey DV, DiFiglia M, Aronin N, Khvorova A. Exosome-mediated Delivery of Hydrophobically Modified siRNA for Huntingtin mRNA Silencing. Mol Ther. 2016 10; 24(10):1836-1847.
    View in: PubMed
    Score: 0.120
  13. 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: 0.119
  14. 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: 0.105
  15. 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: 0.094
  16. Aronin N, Moore M. Hunting down huntingtin. N Engl J Med. 2012 11 01; 367(18):1753-4.
    View in: PubMed
    Score: 0.093
  17. Sah DW, Aronin N. Oligonucleotide therapeutic approaches for Huntington disease. J Clin Invest. 2011 Feb; 121(2):500-7.
    View in: PubMed
    Score: 0.082
  18. 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: 0.073
  19. 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: 0.066
  20. 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: 0.052
  21. 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: 0.048
  22. 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: 0.046
  23. 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: 0.044
  24. 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: 0.044
  25. 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: 0.037
  26. 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: 0.036
  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: 0.033
  28. Block-Galarza J, Chase KO, Sapp E, Vaughn KT, Vallee RB, DiFiglia M, Aronin N. Fast transport and retrograde movement of huntingtin and HAP 1 in axons. Neuroreport. 1997 Jul 07; 8(9-10):2247-51.
    View in: PubMed
    Score: 0.032
  29. Nikan M, Osborn MF, Coles AH, Biscans A, Godinho BMDC, Haraszti RA, Sapp E, Echeverria D, DiFiglia M, Aronin N, Khvorova A. Synthesis and Evaluation of Parenchymal Retention and Efficacy of a Metabolically Stable O-Phosphocholine-N-docosahexaenoyl-l-serine siRNA Conjugate in Mouse Brain. Bioconjug Chem. 2017 06 21; 28(6):1758-1766.
    View in: PubMed
    Score: 0.032
  30. 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: 0.030
  31. 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: 0.030
  32. Choudhury SR, Harris AF, Cabral DJ, Keeler AM, Sapp E, Ferreira JS, Gray-Edwards HL, Johnson JA, Johnson AK, Su Q, Stoica L, DiFiglia M, Aronin N, Martin DR, Gao G, Sena-Esteves M. Widespread Central Nervous System Gene Transfer and Silencing After Systemic Delivery of Novel AAV-AS Vector. Mol Ther. 2016 Apr; 24(4):726-35.
    View in: PubMed
    Score: 0.029
  33. 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: 0.029
  34. 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: 0.027
  35. Zhu LJ, Holmes BR, Aronin N, Brodsky MH. CRISPRseek: a bioconductor package to identify target-specific guide RNAs for CRISPR-Cas9 genome-editing systems. PLoS One. 2014; 9(9):e108424.
    View in: PubMed
    Score: 0.026
  36. 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: 0.025
  37. 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: 0.025
  38. 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: 0.024
  39. 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: 0.022
  40. 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: 0.019
  41. 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: 0.019
  42. Li X, Standley C, Sapp E, Valencia A, Qin ZH, Kegel KB, Yoder J, Comer-Tierney LA, Esteves M, Chase K, Alexander J, Masso N, Sobin L, Bellve K, Tuft R, Lifshitz L, Fogarty K, Aronin N, DiFiglia M. Mutant huntingtin impairs vesicle formation from recycling endosomes by interfering with Rab11 activity. Mol Cell Biol. 2009 Nov; 29(22):6106-16.
    View in: PubMed
    Score: 0.019
  43. 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: 0.018
  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: 0.015
  45. Kegel KB, Sapp E, Yoder J, Cuiffo B, Sobin L, Kim YJ, Qin ZH, Hayden MR, Aronin N, Scott DL, Isenberg G, Goldmann WH, DiFiglia M. Huntingtin associates with acidic phospholipids at the plasma membrane. J Biol Chem. 2005 Oct 28; 280(43):36464-73.
    View in: PubMed
    Score: 0.014
  46. 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: 0.013
  47. 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: 0.012
  48. Kim M, Roh JK, Yoon BW, Kang L, Kim YJ, Aronin N, DiFiglia M. Huntingtin is degraded to small fragments by calpain after ischemic injury. Exp Neurol. 2003 Sep; 183(1):109-15.
    View in: PubMed
    Score: 0.012
  49. 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: 0.011
  50. 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
  51. 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: 0.011
  52. 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: 0.010
  53. Kim M, Lee HS, LaForet G, McIntyre C, Martin EJ, Chang P, Kim TW, Williams M, Reddy PH, Tagle D, Boyce FM, Won L, Heller A, Aronin N, DiFiglia M. Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. J Neurosci. 1999 Feb 01; 19(3):964-73.
    View in: PubMed
    Score: 0.009
  54. 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: 0.009
  55. 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
  56. Velier J, Kim M, Schwarz C, Kim TW, Sapp E, Chase K, Aronin N, DiFiglia M. Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways. Exp Neurol. 1998 Jul; 152(1):34-40.
    View in: PubMed
    Score: 0.009
  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: 0.008
  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: 0.008
  59. 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: 0.008
Connection Strength

The connection strength for concepts is the sum of the scores for each matching publication.

Publication scores are based on many factors, including how long ago they were written and whether the person is a first or senior author.