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N-Terminal Fragments of Huntingtin Longer than Residue 170 form Visible Aggregates Independently to Polyglutamine Expansion.

Chen MZ, Mok SA, Ormsby AR, Muchowski PJ, Hatters DM.

J Huntingtons Dis. 2017;6(1):79-91. doi: 10.3233/JHD-160207.


Adaptive and Behavioral Changes in Kynurenine 3-Monooxygenase Knockout Mice: Relevance to Psychotic Disorders.

Erhardt S, Pocivavsek A, Repici M, Liu XC, Imbeault S, Maddison DC, Thomas MAR, Smalley JL, Larsson MK, Muchowski PJ, Giorgini F, Schwarcz R.

Biol Psychiatry. 2017 Nov 15;82(10):756-765. doi: 10.1016/j.biopsych.2016.12.011. Epub 2016 Dec 16.


Tryptophan 2,3-dioxygenase and indoleamine 2,3-dioxygenase 1 make separate, tissue-specific contributions to basal and inflammation-induced kynurenine pathway metabolism in mice.

Larkin PB, Sathyasaikumar KV, Notarangelo FM, Funakoshi H, Nakamura T, Schwarcz R, Muchowski PJ.

Biochim Biophys Acta. 2016 Nov;1860(11 Pt A):2345-2354. doi: 10.1016/j.bbagen.2016.07.002. Epub 2016 Jul 5.


αB-Crystallin overexpression in astrocytes modulates the phenotype of the BACHD mouse model of Huntington's disease.

Oliveira AO, Osmand A, Outeiro TF, Muchowski PJ, Finkbeiner S.

Hum Mol Genet. 2016 May 1;25(9):1677-89. doi: 10.1093/hmg/ddw028. Epub 2016 Feb 26.


Targeted deletion of kynurenine 3-monooxygenase in mice: a new tool for studying kynurenine pathway metabolism in periphery and brain.

Giorgini F, Huang SY, Sathyasaikumar KV, Notarangelo FM, Thomas MA, Tararina M, Wu HQ, Schwarcz R, Muchowski PJ.

J Biol Chem. 2013 Dec 20;288(51):36554-66. doi: 10.1074/jbc.M113.503813. Epub 2013 Nov 4.


Cannabinoid receptor 2 signaling in peripheral immune cells modulates disease onset and severity in mouse models of Huntington's disease.

Bouchard J, Truong J, Bouchard K, Dunkelberger D, Desrayaud S, Moussaoui S, Tabrizi SJ, Stella N, Muchowski PJ.

J Neurosci. 2012 Dec 12;32(50):18259-68. doi: 10.1523/JNEUROSCI.4008-12.2012.


Mutant huntingtin impairs immune cell migration in Huntington disease.

Kwan W, Träger U, Davalos D, Chou A, Bouchard J, Andre R, Miller A, Weiss A, Giorgini F, Cheah C, Möller T, Stella N, Akassoglou K, Tabrizi SJ, Muchowski PJ.

J Clin Invest. 2012 Dec;122(12):4737-47. doi: 10.1172/JCI64484. Epub 2012 Nov 19.


Genetic Deficiency of Complement Component 3 Does Not Alter Disease Progression in a Mouse Model of Huntington's Disease.

Larkin PB, Muchowski PJ.

J Huntingtons Dis. 2012;1(1):107-18. doi: 10.3233/JHD-2012-120021.


Methylene blue modulates huntingtin aggregation intermediates and is protective in Huntington's disease models.

Sontag EM, Lotz GP, Agrawal N, Tran A, Aron R, Yang G, Necula M, Lau A, Finkbeiner S, Glabe C, Marsh JL, Muchowski PJ, Thompson LM.

J Neurosci. 2012 Aug 8;32(32):11109-19.


Detection of Mutant Huntingtin Aggregation Conformers and Modulation of SDS-Soluble Fibrillar Oligomers by Small Molecules.

Sontag EM, Lotz GP, Yang G, Sontag CJ, Cummings BJ, Glabe CG, Muchowski PJ, Thompson LM.

J Huntingtons Dis. 2012;1(1):119-32. doi: 10.3233/JHD-2012-129004.


Kynurenines in the mammalian brain: when physiology meets pathology.

Schwarcz R, Bruno JP, Muchowski PJ, Wu HQ.

Nat Rev Neurosci. 2012 Jul;13(7):465-77. doi: 10.1038/nrn3257. Review.


Drosophila eye color mutants as therapeutic tools for Huntington disease.

Green EW, Campesan S, Breda C, Sathyasaikumar KV, Muchowski PJ, Schwarcz R, Kyriacou CP, Giorgini F.

Fly (Austin). 2012 Apr-Jun;6(2):117-20. doi: 10.4161/fly.19999. Epub 2012 Apr 1.


Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein.

Nucifora LG, Burke KA, Feng X, Arbez N, Zhu S, Miller J, Yang G, Ratovitski T, Delannoy M, Muchowski PJ, Finkbeiner S, Legleiter J, Ross CA, Poirier MA.

J Biol Chem. 2012 May 4;287(19):16017-28. doi: 10.1074/jbc.M111.252577. Epub 2012 Mar 20.


Suppression of α-synuclein toxicity and vesicle trafficking defects by phosphorylation at S129 in yeast depends on genetic context.

Sancenon V, Lee SA, Patrick C, Griffith J, Paulino A, Outeiro TF, Reggiori F, Masliah E, Muchowski PJ.

Hum Mol Genet. 2012 Jun 1;21(11):2432-49. doi: 10.1093/hmg/dds058. Epub 2012 Feb 21.


Bone marrow transplantation confers modest benefits in mouse models of Huntington's disease.

Kwan W, Magnusson A, Chou A, Adame A, Carson MJ, Kohsaka S, Masliah E, Möller T, Ransohoff R, Tabrizi SJ, Björkqvist M, Muchowski PJ.

J Neurosci. 2012 Jan 4;32(1):133-42. doi: 10.1523/JNEUROSCI.4846-11.2012.


Identifying polyglutamine protein species in situ that best predict neurodegeneration.

Miller J, Arrasate M, Brooks E, Libeu CP, Legleiter J, Hatters D, Curtis J, Cheung K, Krishnan P, Mitra S, Widjaja K, Shaby BA, Lotz GP, Newhouse Y, Mitchell EJ, Osmand A, Gray M, Thulasiramin V, Saudou F, Segal M, Yang XW, Masliah E, Thompson LM, Muchowski PJ, Weisgraber KH, Finkbeiner S.

Nat Chem Biol. 2011 Oct 30;7(12):925-34. doi: 10.1038/nchembio.694. Erratum in: Nat Chem Biol. 2012 Mar;8(3):318.


Expanded polyglutamine-binding peptoid as a novel therapeutic agent for treatment of Huntington's disease.

Chen X, Wu J, Luo Y, Liang X, Supnet C, Kim MW, Lotz GP, Yang G, Muchowski PJ, Kodadek T, Bezprozvanny I.

Chem Biol. 2011 Sep 23;18(9):1113-25. doi: 10.1016/j.chembiol.2011.06.010.


Kynurenine 3-monooxygenase inhibition in blood ameliorates neurodegeneration.

Zwilling D, Huang SY, Sathyasaikumar KV, Notarangelo FM, Guidetti P, Wu HQ, Lee J, Truong J, Andrews-Zwilling Y, Hsieh EW, Louie JY, Wu T, Scearce-Levie K, Patrick C, Adame A, Giorgini F, Moussaoui S, Laue G, Rassoulpour A, Flik G, Huang Y, Muchowski JM, Masliah E, Schwarcz R, Muchowski PJ.

Cell. 2011 Jun 10;145(6):863-74. doi: 10.1016/j.cell.2011.05.020.


The kynurenine pathway modulates neurodegeneration in a Drosophila model of Huntington's disease.

Campesan S, Green EW, Breda C, Sathyasaikumar KV, Muchowski PJ, Schwarcz R, Kyriacou CP, Giorgini F.

Curr Biol. 2011 Jun 7;21(11):961-6. doi: 10.1016/j.cub.2011.04.028.


Functional gene expression profiling in yeast implicates translational dysfunction in mutant huntingtin toxicity.

Tauber E, Miller-Fleming L, Mason RP, Kwan W, Clapp J, Butler NJ, Outeiro TF, Muchowski PJ, Giorgini F.

J Biol Chem. 2011 Jan 7;286(1):410-9. doi: 10.1074/jbc.M110.101527. Epub 2010 Nov 2.


Targeting kynurenine 3-monooxygenase (KMO): implications for therapy in Huntington's disease.

Thevandavakkam MA, Schwarcz R, Muchowski PJ, Giorgini F.

CNS Neurol Disord Drug Targets. 2010 Dec;9(6):791-800. Review.


Hsp70 and Hsp40 functionally interact with soluble mutant huntingtin oligomers in a classic ATP-dependent reaction cycle.

Lotz GP, Legleiter J, Aron R, Mitchell EJ, Huang SY, Ng C, Glabe C, Thompson LM, Muchowski PJ.

J Biol Chem. 2010 Dec 3;285(49):38183-93. doi: 10.1074/jbc.M110.160218. Epub 2010 Sep 23.


Dysfunctional kynurenine pathway metabolism in the R6/2 mouse model of Huntington's disease.

Sathyasaikumar KV, Stachowski EK, Amori L, Guidetti P, Muchowski PJ, Schwarcz R.

J Neurochem. 2010 Jun;113(6):1416-25. doi: 10.1111/j.1471-4159.2010.06675.x. Epub 2010 Mar 17.


Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo.

Legleiter J, Mitchell E, Lotz GP, Sapp E, Ng C, DiFiglia M, Thompson LM, Muchowski PJ.

J Biol Chem. 2010 May 7;285(19):14777-90. doi: 10.1074/jbc.M109.093708. Epub 2010 Mar 10.


Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease.

Sathasivam K, Lane A, Legleiter J, Warley A, Woodman B, Finkbeiner S, Paganetti P, Muchowski PJ, Wilson S, Bates GP.

Hum Mol Genet. 2010 Jan 1;19(1):65-78. doi: 10.1093/hmg/ddp467.


Polyglutamine dances the conformational cha-cha-cha.

Miller J, Rutenber E, Muchowski PJ.

Structure. 2009 Sep 9;17(9):1151-3. doi: 10.1016/j.str.2009.08.004.


Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease.

Wacker JL, Huang SY, Steele AD, Aron R, Lotz GP, Nguyen Q, Giorgini F, Roberson ED, Lindquist S, Masliah E, Muchowski PJ.

J Neurosci. 2009 Jul 15;29(28):9104-14. doi: 10.1523/JNEUROSCI.2250-09.2009.


Genetic manipulation of palmitoylethanolamide production and inactivation in Saccharomyces cerevisiae.

Muccioli GG, Sia A, Muchowski PJ, Stella N.

PLoS One. 2009 Jun 16;4(6):e5942. doi: 10.1371/journal.pone.0005942.


Exploiting yeast genetics to inform therapeutic strategies for Huntington's disease.

Giorgini F, Muchowski PJ.

Methods Mol Biol. 2009;548:161-74. doi: 10.1007/978-1-59745-540-4_9.


Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment.

Legleiter J, Lotz GP, Miller J, Ko J, Ng C, Williams GL, Finkbeiner S, Patterson PH, Muchowski PJ.

J Biol Chem. 2009 Aug 7;284(32):21647-58. doi: 10.1074/jbc.M109.016923. Epub 2009 Jun 2.


Of mice, rats and men: Revisiting the quinolinic acid hypothesis of Huntington's disease.

Schwarcz R, Guidetti P, Sathyasaikumar KV, Muchowski PJ.

Prog Neurobiol. 2010 Feb 9;90(2):230-45. doi: 10.1016/j.pneurobio.2009.04.005. Epub 2009 Apr 24. Review.


Induction of the phase II detoxification pathway suppresses neuron loss in Drosophila models of Parkinson's disease.

Trinh K, Moore K, Wes PD, Muchowski PJ, Dey J, Andrews L, Pallanck LJ.

J Neurosci. 2008 Jan 9;28(2):465-72. doi: 10.1523/JNEUROSCI.4778-07.2008.


Noninvasive measurement of protein aggregation by mutant huntingtin fragments or alpha-synuclein in the lens.

Muchowski PJ, Ramsden R, Nguyen Q, Arnett EE, Greiling TM, Anderson SK, Clark JI.

J Biol Chem. 2008 Mar 7;283(10):6330-6. doi: 10.1074/jbc.M709678200. Epub 2007 Dec 31.


Histone deacetylase inhibition modulates kynurenine pathway activation in yeast, microglia, and mice expressing a mutant huntingtin fragment.

Giorgini F, Möller T, Kwan W, Zwilling D, Wacker JL, Hong S, Tsai LC, Cheah CS, Schwarcz R, Guidetti P, Muchowski PJ.

J Biol Chem. 2008 Mar 21;283(12):7390-400. Epub 2007 Dec 13.


Accelerating amyloid-beta fibrillization reduces oligomer levels and functional deficits in Alzheimer disease mouse models.

Cheng IH, Scearce-Levie K, Legleiter J, Palop JJ, Gerstein H, Bien-Ly N, Puoliväli J, Lesné S, Ashe KH, Muchowski PJ, Mucke L.

J Biol Chem. 2007 Aug 17;282(33):23818-28. Epub 2007 Jun 4.


Chaperone functions of the E3 ubiquitin ligase CHIP.

Rosser MF, Washburn E, Muchowski PJ, Patterson C, Cyr DM.

J Biol Chem. 2007 Aug 3;282(31):22267-77. Epub 2007 Jun 1.


Screening for genetic modifiers of amyloid toxicity in yeast.

Giorgini F, Muchowski PJ.

Methods Enzymol. 2006;412:201-22. Review.


Disease-modifying pathways in neurodegeneration.

Finkbeiner S, Cuervo AM, Morimoto RI, Muchowski PJ.

J Neurosci. 2006 Oct 11;26(41):10349-57. Review. No abstract available.


Green tea (-)-epigallocatechin-gallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington's disease models.

Ehrnhoefer DE, Duennwald M, Markovic P, Wacker JL, Engemann S, Roark M, Legleiter J, Marsh JL, Thompson LM, Lindquist S, Muchowski PJ, Wanker EE.

Hum Mol Genet. 2006 Sep 15;15(18):2743-51. Epub 2006 Aug 7.


Flanking sequences profoundly alter polyglutamine toxicity in yeast.

Duennwald ML, Jagadish S, Muchowski PJ, Lindquist S.

Proc Natl Acad Sci U S A. 2006 Jul 18;103(29):11045-50. Epub 2006 Jul 10.


A network of protein interactions determines polyglutamine toxicity.

Duennwald ML, Jagadish S, Giorgini F, Muchowski PJ, Lindquist S.

Proc Natl Acad Sci U S A. 2006 Jul 18;103(29):11051-6. Epub 2006 Jul 10.


A genomic screen in yeast implicates kynurenine 3-monooxygenase as a therapeutic target for Huntington disease.

Giorgini F, Guidetti P, Nguyen Q, Bennett SC, Muchowski PJ.

Nat Genet. 2005 May;37(5):526-31. Epub 2005 Apr 3.


Connecting the dots in Huntington's disease with protein interaction networks.

Giorgini F, Muchowski PJ.

Genome Biol. 2005;6(3):210. Epub 2005 Feb 28. Review.


Modulation of neurodegeneration by molecular chaperones.

Muchowski PJ, Wacker JL.

Nat Rev Neurosci. 2005 Jan;6(1):11-22. Review.


Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer.

Wacker JL, Zareie MH, Fong H, Sarikaya M, Muchowski PJ.

Nat Struct Mol Biol. 2004 Dec;11(12):1215-22. Epub 2004 Nov 14.


Molecular genetics approaches in yeast to study amyloid diseases.

Outeiro TF, Muchowski PJ.

J Mol Neurosci. 2004;23(1-2):49-60. Review.


Yeast genes that enhance the toxicity of a mutant huntingtin fragment or alpha-synuclein.

Willingham S, Outeiro TF, DeVit MJ, Lindquist SL, Muchowski PJ.

Science. 2003 Dec 5;302(5651):1769-72.


Cysteine string protein (CSP) inhibition of N-type calcium channels is blocked by mutant huntingtin.

Miller LC, Swayne LA, Chen L, Feng ZP, Wacker JL, Muchowski PJ, Zamponi GW, Braun JE.

J Biol Chem. 2003 Dec 26;278(52):53072-81. Epub 2003 Oct 21.


Making yeast tremble: yeast models as tools to study neurodegenerative disorders.

Sherman MY, Muchowski PJ.

Neuromolecular Med. 2003;4(1-2):133-46. Review.


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