The Muchowski Lab
Molecular Mechanisms of Neurodegenerative
Disorders Associated with Protein Misfolding



 

Publications

2011

Daniel Zwilling,  Shao-Yi Huang, Korrapati V. Sathyasaikumar, Francesca M. Notarangelo, Paolo Guidetti, Hui-Qiu Wu, Jason Lee, Jennifer Truong, Yaisa Andrews-Zwilling, Eric W. Hsieh, Jamie Y. Louie, Tiffany Wu, Kimberly Scearce-Levie, Christina Patrick, Anthony Adame, Flaviano Giorgini, Saliha Moussaoui, Grit Laue, Arash Rassoulpour, Gunnar Flik, Yadong Huang, Joseph M. Muchowski, Eliezer Masliah, Robert Schwarcz, and Paul J. Muchowski, "Kynurenine 3-Monooxygenase Inhibition in Blood Ameliorates Neurodegeneration" (Full Text)

Peter H. Reinhart and Jeffery W. Kelly, "Treating the Periphery to Ameliorate Neurodegenerative Diseases",   Cell 145, June 10, 2011. (Full Text)

Xuesong Chen, Jun Wu, Yuan Luo, Xia Liang, Charlene Supnet, Mee Whi Kim, Gregor P. Lotz, Guocheng Yang,, Paul J. Muchowski, Thomas Kodadek, and Ilya Bezprozvanny, "Expanded Polyglutamine-Binding Peptoid as a Novel Therapeutic Agent for Treatment of Huntington’s Disease", (Full Text)

Jason Miller, Montserrat Arrasate, Elizabeth Brooks, Clare Peters Libeu, Justin Legleiter, Danny Hatters, Jessica Curtis, Kenneth Cheung, Preethi Krishnan, Siddhartha Mitra, Kartika Widjaja, Benjamin AShaby, Gregor PLotz, Yvonne Newhouse, Emily J Mitchell, Alex Osmand, Michelle Gray, Vanitha Thulasiramin, Frédéric Saudou, Mark Segal, X William Yang, Eliezer Masliah, Leslie M Thompson, Paul J Muchowski, Karl H Weisgraber & Steven Finkbeiner, "Identifying polyglutamine protein species in situ that best predict neurodegeneration", National Chemical Biology,  published online: 30 october 2011 | doi: 10.1038/NChemBio.694. (Full Text)

2010

Eran Tauber, Leonor Miller-Fleming, Robert P. Mason, Wanda Kwan, Jannine Clapp, Nicola J. Butler, Tiago F. Outeiro, Paul J. Muchowski, and Flaviano Giorgini, "Functional Gene Expression Profiling in Yeast Implicates Translational Dysfunction in Mutant Huntingtin Toxicity", (Full Text)

Gregor P. Lotz, Justin Legleiter, Rebecca Aron, Emily J. Mitchell, Shao-Yi Huang, Cheping Ng, Charles Glabe, Leslie M. Thompson, and Paul J. Muchowski, "Hsp70 and Hsp40 Functionally Interact with Soluble Mutant Huntingtin Oligomers in a Classic ATP-dependent Reaction Cycle",  (Full Text)

Robert Schwarcz, Paolo Guidetti, Korrapati V. Sathyasaikumar, Paul J. Muchowski, "Of mice, rats and men: Revisiting the quinolinic acid hypothesis of Huntington’s disease",  Progress in Neurobiology, Special Issue Chemical Signaling in the Nervous System in Health and Disease: Nils-Ake Hillarp's Legacy, published by Elsevier. (Full Text)

2009

Sathasivam K, Lane A, Legleiter J, Warley A, Woodman B, Finkbeiner S, Paganetti P, Muchowski PJ, Wilson S, Bates GP., "Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease", Hum. Mol Genet.,(2009). (Full Text)

Miller J, Rutenber E, Muchowski PJ., "Polyglutamine dances the conformational cha-cha-cha.", Structure, 17(9):1151-3(2009). (Full Text)

Wacker JL, Huang SY, Steele AD, Aron R, Lotz GP, Nguyen Q, Giorgini F, Roberson ED, Lindquist S, Masliah E, Muchowski PJ., "Loss of Hsp70 exacerbates pathogenesis but not levels of fibrillar aggregates in a mouse model of Huntington's disease.", J. Neurosci., 29(28):9104-14 (2009). (Full Text)

Muccioli GG, Sia A, Muchowski PJ, Stella N., "Genetic manipulation of palmitoylethanolamide production and inactivation in Saccharomyces cerevisiae.", PLoS One, 4(6):e5942. (2009). (Full Text)

Giorgini F, Muchowski PJ., "Exploiting yeast genetics to inform therapeutic strategies for Huntington's disease.", Methods Mol. Biol.,, 548:161-74 (2009). (Full Text)

Legleiter J, Lotz GP, Miller J, Ko J, Ng C, Williams GL, Finkbeiner S, Patterson PH, Muchowski PJ., "Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment.", J. Neurosci., 284(32):21647-58 (2009). (Full Text)

Salazar L, Kashiwada T, Krejci P, Muchowski P, Donoghue D, Wilcox WR, Thompson LM., "A novel interaction between fibroblast growth factor receptor 3 and the p85 subunit of phosphoinositide 3-kinase: activation-dependent regulation of ERK by p85 in multiple myeloma cells.", J. Biol. Chem.,, 284(32):21647-58 (2009). (Full Text)

2008

Trinh, K., Moore, K., Wes, P.D., Muchowski, P.J., Dey, J., Andrews, L., Pallanck, L.J., "Induction of the phase II detoxification pathway suppresses neuron loss in Drosophila models of Parkinson's disease", J. Neurosci., 28: 465-472 (2008). (Full Text)

Muchwoski, P.J., Ramsden, R., Nguyen, Q., Arnett, E.E., Greiling, T.M., Anderson, S.K., Clark, J.I., "Noninvasive measurement of protein aggregation by mutant huntingtin fragments or alpha-synuclein in the lens", J. Biol. Chem., 283: 6330-6336 (2008). (Full Text)

Giorgini, F., Moller, T., Kwan, W., Zwilling, D., Wacker, J.L., Hong, S., Tsai, L.C., Cheah, C.S., Schwarcz, R., Guidetti, P., Muchowski, P.J. "Histone deacetylase inhibition modulates kynurenine pathway activation in yeast, microglia, and mice expressing a mutant huntingtin fragment", J. Biol. Chem., 283: 7390-7400 (2008). (Full Text)

2007

Irene H. Cheng, Kimberly Scearce-Levie, Justin Legleiter, Jorge J. Palop, Hilary Gerstein, Nga Bien-Ly, Jukka Puoliva¨ li, Sylvain Lesne´ , Karen H. Ashe, Paul J. Muchowski, and Lennart Mucke, "Accelerating Amyloid-Fibrillization Reduces Oligomer Levels and Functional Deficits in Alzheimer Disease Mouse Models,  From the Gladstone Institute of Neurological Disease, San Francisco, California 94158, the Departments of Neurology and Biochemistry and Biophysics, University of California, San Francisco, California 94143, and the Department of Neurology, University of Minnesota Medical School, Minneapolis, Minnesota 55455  (Full Text)

Cheng, I.H., Scearce-Levie, K., Legleiter, J., Palop, J.J., Gerstein, H., Nga, B.L., Puoliväli, J., Lesné, S., Ashe, K.H., Muchowski, P.J., and Mucke, L., “Enhancing the fibrillization and deposition of the amyloid-b peptide reduces its negative impact on behavior and synaptic activity-dependent proteins”, J. Biol. Chem., 282:23818-23828 (2007). (Full Text)

Rosser, M.F.N., Muchowski, P.J., Patterson, C. and Cyr, D.M., “Chaperone Functions of the E3 Ubiquitin Ligase CHIP”, J. Biol. Chem., 282:22267-22277 (2007). (Full Text)
 

2006

Dagmar E. Ehrnhoefer, Martin Duennwald, Phoebe Markovic, Jennifer L. Wacker, Sabine Engemann, Margaret Roark, Justin Legleiter, J. Lawrence Marsh, Leslie M. Thompson, Susan Lindquist, Paul J. Muchowski and Erich E. Wanker. "Green tea (2)-epigallocatechin-gallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington’s disease models", Human Molecular Genetics, 2006, Vol. 15, No. 18 2743–2751 (Full Text)

Giorgini, F. and Muchowski, P.J., “Screening for genetic modifiers of amyloid toxicity in yeast”, Methods Enzymol., 412:201-22 (2006). (Full Text)

Finkbeiner, S., Cuervo, A.M., Morimoto, R.I. and Muchowski, P.J., “Disease-modifying pathways in neurodegeneration”, J. Neurosci., 26:10349-57 (2006). (Full Text)

Duennwald, M., Jagadish, S., Muchowski, P.J. and Lindquist, S., “Flanking sequences profoundly alter polyglutamine toxicity in yeast”, Proc. Natl. Acad. Sci. U.S.A., 103:11,045-11,050, 2006. (Full Text)

 Duennwald, M., Jagadish, S., Giorgini, F., Muchowski, P.J. and Lindquist, S., “A network of protein interactions determines polyglutamine toxicity”, Proc. Natl. Acad. Sci. U.S.A., 103:11,051-11,056, 2006. (Full Text)

 

2005

Giorgini, F., Guidetti, P., Nguyen, Q.V., Bennett, S.C. and Muchowski, P.J., “A genomic screen in yeast implicates kynurenine 3-monooxygenase as a therapeutic target for Huntington’s disease”, Nature Genetics, 37:526-531, 2005. (Full Text)

Giorgini, F. and Muchowski, P.J., “Connecting the dots in Huntington’s disease: exploiting protein interaction networks”, Genome Biology, 6:210, 2005. (Full Text) 

Muchowski, P.J. and Wacker, J.L., “Modulation of neurodegeneration by molecular chaperones” Nat. Rev. Neurosci., 6:11-22, 2005. (Full Text)

 

2004

Wacker, J.L., Zareie, M.H., Fong, H., Sarikaya, M. and Muchowski, P.J., “Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer”, Nat. Struct. and Mol. Biol., 11:1101-1106, 2004. (Full Text)

Tiago Fleming Outeiro,1 and Paul J. Muchowski, "Molecular Genetics Approaches in Yeast to Study Amyloid Diseases",  Journal of Molecular Neuroscience Review Article Volume 23 Nos. 1–2, 2004. (Full Text)

 

2003

Willingham, S., Outeiro, T.F., DeVit, M.J., Lindquist, S.L. and Muchowski, P.J., “Yeast genes that enhance the toxicity of a mutant huntingtin fragment or a-synuclein”, Science, 302:1769-1772, 2003. (Full Text)

 

2002

Muchowski, P.J., “Protein misfolding, amyloid formation and neurodegeneration: A critical role for molecular chaperones?”, Neuron, 35:9-12, 2002.  (Full Text)

 
 

 

Gladstone Institute of Neurological Disease
University of California San Francisco

1650 Owens Street
San Francisco, CA 94158
Telephone: 415-734-2516