Langfelder P., Gao F., Wang N., Howland D., Kwak S., Vogt T.F., Aaronson J.S., Rosinski J., Coppola G., Horvath S., Yang X.Y. (2018). MicroRNA signatures of endogenous Huntingtin CAG repeat expansion in mice. PLoS One.  13(1): e0190550.


Victor M.B., Richner M., Olsen H.E., Lee S.W., Monteys A.M., Ma C., Huh C.J., Zhang B., Davidson B.L., Yang X.W., Yoo A.S. (2018). Striatal neurons directly converted from Huntington’s disease patient fibroblasts recapitulate age-associated disease phenotypes. Nature Neurosci. [Epub ahead of print]


Veldman M.B., Yang X.W. (2018).  Molecular insights into cortico-striatal miscommunications in Huntington’s disease. Curr Opin Neurobiol. 48: 79-89.



Yau R.G., Doerner K., Castellanos E.R., Haakonsen D.L., Werner A., Wang N., Yang X.W., Martinez-Martin N., Matsumoto M.L., Dixit V.M., Rape M. (2017).  Assembly and function of heterotypic ubiquitin chains in cell-cycle and protein quality control.  Cell.  171(4): 918-933.e20.


Veldman M.B., Yang X.W. (2017).  Huntington’s disease: nuclear gatekeepers under attack.  Neuron. 94: 1-4.


Lu X.H., Yang X.W. (2017).  Genetically-directed sparse neuronal labeling in BAC transgenic mice through mononucleotide repeat frameshift. Sci Rep. 7: 43915.


Southwell A.L., Skotte N.H., Villaneuva E.B., Østergaard M.E., Gu X., Kordasiewicz H.B., Kay C., Cheung D., Yuanyun X., Waltl S., Del Cengio L., Findlay-Black H., Doty C.N., Petoukhov E., Iworima D., Slama R., Ooi J., Pouladi M.A., Yang X.W., Swayze E.E., Seth P.P., Hayden M.R. (2017). A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles. Hum Mol Genet. 26(6): 1115-1132.



Yang X.W. (2016). Life and death rest on a bivalent chromatin state. Nature Neurosci. 19(10): 1271-3.


Hintiryan H., Foster N.N., Bowman I., Bay M., Song M.Y., Gou L., Yamashita S., Bienkowski M.S., Zingg B., Zhu M., Yang X.W., Shih J.C., Toga A.W., Dong H.W. (2016). The mouse cortico-striatal projectome. Nature Neurosci. 19: 1100-14.


Horvath S., Langfelder P., Kwak S., Aaronson J., Rosinski J., Vogt T.F., Eszes M., Faull R.L., Curtis M.A., Waldvogel H.J., Choi O.W., Tung S., Vinters H.V., Coppola G., Yang X.W. (2016). Huntington’s disease accelerates epigenetic aging of human brain and disrupts DNA methylation levels. Aging (Albany NY). 8: 1485-512.


Kratter, I.H., Zahed H., Lau A., Tsvetkov A.S., Daub A.C., Weiberth K.F., Gu X., Saudou F., Humbert S., Yang X.W., Osmand A., Steffan J.S., Masliah E., Finkbeiner S. (2016). Serine 421 regulates mutant huntingtin toxicity and clearance in mice. J Clin Invest. 126(9): 3585-97.


Xu, J., Bernstein, A., Wong, A., Lu, X., Khoja, S., Yang, X.W., Davies, D.L., Micevych, P., Sofroniew, M.V., Khakh, B.S. (2016). P2X4 Receptor Reporter Mice: Sparse Brain Expression and Feeding-Related Presynaptic Facilitation in the Arcuate Nucleus. J Neuroscience. 36: 8902-8920.


Chandra, A., Sharma, A., Calingasan, N.Y., White, J.M., Shurubor, Y., Yang, X.W., Beal, M.F. and Johri, A. (2016). Enhanced Mitochondrial Biogenesis Ameliorates Disease Phenotype in a Full-Length Mouse Model of Huntington’s Disease. Hum Mol Gen. 25(11): 2269-2282.


Langfelder, P., Cantle, J.P., Chatzopoulou, D., Wang, N., Gao, F., Al-Ramahi, I., Lu, X.H., Ramos, E.M., El-Zein, K., Zhao, Y., Deverasetty, S., Tebbe, A., Schaab, C., Lavery, D.J., Howland, D., Kwak, S., Botas, J., Aaronson, J.S., Rosinski, J., Coppola, G., Horvath, S., and Yang, X.W. (2016). Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice. Nature Neurosci. 19: 623-633.



Veldman, M.B., Rios-Galdamez, Y., Lu, X.H., Gu, X., Qin, W., Li, S., Yang, X.W. and Lin, S. (2015). The N17 domain mitigates nuclear toxicity in a novel zebrafish Huntington’s disease model. Mol Neurodegener.. 10: 1-16.


Park, C.S. and Yang, X.W. (2015). Probing the stress and depression circuits with a disease gene. eLife. 4:e10829.


Estrada-Sanchez, A., Burroughs, C., Cavaliere, S., Barton, S., Chen, S., Yang, X., and Rebec, G. (2015). Cortical Efferents Lacking Mutant huntingtin Improve Striatal Neuronal Activity and Behavior in a Conditional Mouse Model of Huntington’s Disease. J Neuroscience. 35: 4440-4451.


Gu, X., Cantle, J.P., Greiner, E. C.Y. Lee, C.Y.D., Barth, A.M., Gao, F., Park, C.S., Zhang, Z., Sandoval, S., Zhang, R., Diamond, M., Mody, I., Coppola, G., Yang, X.W. (2015). N17 Modifies Mutant Huntingtin Nuclear Pathogenesis and Severity of Disease in HD BAC Transgenic Mice. Neuron. 85: 726-741.

[UCLA Press Release]   [Striatal Network Browser]   [HD Buzz]


Peñagarikano, O., Lázaro, M.T., Lu, X.H., Gordon, A., Dong, H., Lam, H.A., Peles, E., Maidment, N.T., Murphy, N.P., Yang, X.W., Golshani, P., Geschwind, D.H. (2015). Exogenous and evoked oxytocin restores social behavior in the Cntnap2 mouse model of autism. Sci Transl Med.  271: 271ra8-271ra8



Lu, X.H., Mattis, V.B., Wang, N., Al-Ramahi, I., van den Berg, N., Fratantoni, S.A., Waldvogel, H., Greiner, E., Osmand, A., Elzein, K., Xiao, J., Dijkstra, S., de Pril, R., Vinters, H.V., Faull, R., Signer, E., Kwak, S., Marugan, J.J., Botas, J., Fischer, D.F., Svendsen, C.N., Munoz-Sanjuan, I., Yang, X.W. (2014) Targeting ATM ameliorates mutant Huntingtin toxicity in cell and animal models of Huntington’s disease. Sci Transl Med. 6:268ra178. PMID: 25540325

[ Research Highlight in Nature Reviews Drug Discovery]   [HD Buzz]


Wang, N., Gray, M., Lu, X.H., Cantle, J.P., Holley, S.M., Greiner, E., Gu, X., Shirasaki, D., Cepeda, C., Li, Y., Dong, H.W., Levine, M.S., Yang, X.W. (2014). Neuronal targets of mutant huntingtin genetic reduction to ameliorate Huntington’s disease pathogenesis in mice. Nature Medicine 20:536-541. [Cover Story].

[Nature Medicine Cover]   [Research Highlight in Nature Reviews Neuroscience]   [UCLA Press Release]   [Most Influential HD Research of 2014, HD Insights]   [HD Buzz]


Yang, X.W., Yamamoto A. (2014). CLEARance wars: PolyQ strikes back. Nature Neuroscience 17:1140-1142.


Haustein, M.D., Kracun, S., Lu, X.H., Shih, T., Jackson-Weaver, O., Tong, X., Xu, J., Yang, X.W., O’Dell, T.J., Marvin, J.S., Ellisman, M.H., Bushong, E.A., Looger, L.L., Khakh, B.S. (2014). Conditions and constraints for astrocyte calcium signaling in the hippocampal mossy fiber pathway. Neuron 82:413-429.


Rudnicki, D.D., Yang, X.W. and Margolis, R.L. (2014). HDL2 Mouse. Pp 573-583. In “ Movement Disorders: Genetics and Models. (Edited by Mark S. LeDoux). Academic Press (Elsevier): London, U.K.


Cui, Y., Ostlund, S.B., James, A., Park, C.S., Ge, W., Roberts, K.W., Mittal,N., Murphy, N.P., Cepeda, C., Kieffer, B.L., Levine, M.S., Jentsch, J.D., Walwyn, W.M., Sun, Y.E., Evans, C.J., Maidment, N.T., Yang, X.W. (2014). Targeted expression of m-opioid receptors in a subset of striatal direct-pathway neurons restores opiate reward. Nature Neuroscience 17:254-261.



Wang, N., Lu, X.H., Sandoval, S.V., Yang, X. W.  (2013). An Independent Study of the Preclinical Efficacy of C2-8 in the R6/2 Transgenic Mouse Model of Huntington’s Disease. J. Huntington’s Dis. 2: 443-451.


Lee, C.Y., Cantle, J.P., Yang, X.W. (2013). Genetic manipulations of mutant huntingtin in mice: new insights into Huntington’s disease pathogenesis. FEBS J. 280:4382-4394.
Yue, Z., Yang, X.W. (2013) A dangerous duet: LRRK2 and α-synuclein jam at CMA. Nature Neuroscience 16: 375-377.


Southwell, A.L., Warby, S.C., Carroll, J.B., Doty, C.N., Skotte, N.H., Zhang, W., Villanueva, E.B., Kovalik, V., Xie, Y., Pouladi, M.A., Collins, J.A., Yang, X.W., Franciosi, S., Hayden, M.R. (2013). A fully humanized transgenic mouse model of Huntington disease. Hum Mol Genet. 22:18-34.


Daggett, A., Yang, X. W. (2013) Huntington’s Disease: Easing the NMDAR Traffic Jam.  Nature Medicine 19: 971-973.



Yu-Taeger, L., Petrasch-Parwez, E., Osmand, A., Redensek,A., Metzger, S. Clemens, L., Park,L., Howland, D., Calaminus, C., Gu, X., Pichler, B., Yang, X.W., Riess, O.,  and Nguyen, H.P. (2012). A novel BACHD transgenic rat exhibits characteristic neuropathological features of Huntington disease.  J Neuroscience 32:15426-15438.


Southwell, A.L., Warby, S.C., Carroll, J.B., Doty, C.N., Skotte, N.H., Zhang, W., Villanueva, E.B., Kovalik, V., Xie, Y., Pouladi, M.A., Collins, J.A., Yang, X.W., Franciosi, S. and Hayden, M.R.  (2012). A fully humanized transgenic mouse model of Huntington disease. Hum Mol Genet.


Shirasaki, D.I., Greiner, E.R., Al-Ramahi, I., Gray, M., Boontheung, P., Botas, J., Coppola, G., Horvath, S., Loo, J.A.*, Yang, X.W.* (2012). Network organization of the Huntingtin proteomic interactome in mammalian brain. Neuron 75, 41-57. (*co-corresponding authors)

[Neuron Video Abstract]   [HD Buzz]


Gafni, J., Papanikolaou, T., DeGiacomo, F., Holcomb, J., Chen, S., Menalled, L., Kudwa, A., Fitzpatrick, J., Miller, S., Ramboz, S., Tuunanen, P., Lehtimaki, K., Yang, X.W., Kwak, S., Park, L., Howland, D., Park, H., and Ellerby, L. (2012). Caspase-6 Activity in a BACHD mouse modulates steady-state levels of mutant huntingtin protein but is not necessary for production of a 586 amino acid proteolytic fragment. J Neuroscience 32, 7454-7465.


Cantle, J.P., Lu, X.H., Gu, X.F., Yang, X.W. (2012). Cellular and molecular mechanisms implicated in pathogenesis of selective neurodegeneration in Huntington’s Disease. Frontiers in Biology 7: 459-476.


Lu, X., Yang, X.W. (2012). “Huntingtin Holiday”: Progress towards an antisense therapy for Huntington’s disease. Neuron 74:964-966.



Jiang, M., Wang, J., Fu, J., Du, L., Jeong, H., West, T., Xiang, L., Peng, Q., Hou, Z., Cai, H., Seredenina, T., Arbez, N., Zhu, S., Sommers, K., Qian, J., Zhang, J., Mori, S., Yang, X.W., Tamashiro, K.L., Aja, S., Moran, T.H., Luthi-Carter, R., Martin, B., Maudsley, S., Mattson, M.P., Cichewicz, R.H., Ross, C.A., Holtzman, D.M., Krainc, D., Duan,W. (2011). Neuroprotective role of Sirt1 in mammalian models of Huntington’s disease through activation of multiple Sirt1 targets. Nature Medicine 18, 153-158.


Miller, J., Arrasate, M., Brooks, E., Libeu, C.P., Legleiter, J., Hatters, D., Curtis, J., Cheung, K., Krishnan, P., Mitra, S., Widjaja, K., Shaby, B.A., Lotz, G.P., Newhouse, Y., Mitchell, E.J., Osmand, A., Gray, M., Thulasiramin, V., Saudou., F, Segal, M., Yang, X.W., Masliah, E., Thompson, L.M., Muchowski, P.J., Weisgraber, K.H., Finkbeiner, S. (2011). Identifying polyglutamine protein species in situ that best predict neurodegeneration. Nature Chemical Biology 7, 925-934.


Wilburn, B., Rudnicki, D.D., Zhao, J., Weitz, T.M., Cheng, Y., Gu, X., Greiner, E., Park, C.S., Wang, N., Sopher, B.L., La Spada, A., Osmand, A., Margolis, R.L., Sun, Y.E., Yang, X.W.. (2011). A novel antisense CAG repeat transcript at JPH3 locus mediating expanded polyglutamine protein toxicity in Huntington’s Disease-Like 2 (HDL2) Mice. Neuron 70, 427-440.

[Neuron Preview]   [HDSA Press Release]


Tao, J., Wu, H., Lin, Q., Wei, W., Lu, X.H., Cantle, J.P., Ao, Y., Olsen, R.W., Yang, X.W., Mody, I., Sofroniew, M.V., Sun, Y.E. (2011). Deletion of astroglial dicer causes non-cell-autonomous neuronal dysfunction and degeneration. J Neuroscience 31, 8306-8319.


Yang, X.W. and Lu, X.H. (2011). Molecular and Cellular Basis of OCD-like Behaviors: Emerging View from Mouse Models. Current Opinion in Neurology 24, 114-118.


André, V.M., Cepeda, C., Fisher, Y.E., Huynh, M., Bardakjian, N., Singh, S., Yang, X.W., Levine, M.S. (2011). Differential electrophysiological changes in striatal output neurons in Huntington’s disease. J Neuroscience 31, 1170-1182.


Greiner, E.R., Yang, X.W. (2011). Huntington’s disease: Flipping a switch on huntingtin. Nature Chemical Biology 7, 412-414.



Graham, R.K., Deng, Y., Carroll, J., Vaid, K., Cowan, C., Pouladi, M.A., Metzler, M., Bissada, N., Wang, L., Faull, R.L., Gray, M., Yang, X.W., Raymond, L.A., Hayden, M.R. (2010). Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo. J Neuroscience 30,15019-15029.


André, V.M., Cepeda, C., Cummings, D.M., Jocoy, E.L., Fisher, Y.E., Yang, X.W., Levine, M.S. (2010). Dopamine modulation of excitatory currents in the striatum is dictated by the expression of D1 and D2 receptors and modified by endocannabinoids. Eur J Neurosci. 31, 14-28.


Pouladi, M.A., Xie, Y., Skotte, N.H., Ehrnhoefer, D.E., Graham, R.K., Kim, J.E., Bissada, N., Yang, X.W., Paganetti, P., Friedlander, R.M., Leavitt, B.R., Hayden, M.R.  (2010). Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression. Hum Mol Genet. 19, 1528-1538.


Yang, X.W. and Gray, M. (2010). Mouse model of Huntington’s Disease for validating preclinical candidates. pp 165-196. In “New Therapeutic Development in Huntington’s Disease” (Edited by Don Lo and Robert Hughes). CRC Press. Boca Raton, Florida.



Gu, X., Greiner, E.R., Mishra, R., Kodali, R., Osmand, A., Finkbeiner, S., Steffan, J.S., Thompson, L.M., Wetzel, R., and Yang, X.W. (2009). Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice. Neuron 64:828-840.

[NINDS Press Release]   [UCLA Press Release]   [NBC Nightly News]   [HDSA Report]


Lu, X.H., Fleming, S.M., Meurers, B., Ackerson, C.A., Mortazavi, F., Lo, V., Hernandez, D., Sulzer, Z., Jackson, G.R., Maidment, N.T., Chesselet, M.F., Yang, X.W. (2009). BAC mice with a truncated mutant Parkin exhibit age-dependent hypokinetic motor deficits, dopaminergic neuron degeneration, and accumulation of proteinase K-resistant alpha Synuclein. J. Neuroscience 29, 1962-1976.

[Journal of Neuroscience, Journal Club]


Menalled, L,. El-Khodor, B.F., Patry, M., Suarez-Farinas, M., Orenstein, S., Zahasky, B., Ragland, N., Leahy, C., Yang, X.W., McDonald, M., Morton, J., Bates, J., Signer, E., Tobin, A., Leeds, J., Park, L., Howland, D., and Brunner, D. (2009). Systematic behavioral evaluation of Huntington’s disease transgenic and knock-in mouse models. Neurobiology of Disease 35, 319-336.


Hutnick, L.K., Golshani, P., Namihira, M., Xue, Z., Matynia, A., Yang, X.W., Silva, A.J., Schweizer, F.E., Fan, G. (2009). DNA hypomethylation restricted to the murine forebrain induces cortical degeneration and impairs postnatal neuronal maturation. Hum Mol Genet. 18, 2875-2888.



Yang, X.W. and Lu, X.H. (2008). The BAC transgenic approach to study Parkinson’s disease in mice. pp 247-268. In “Parkinson’s Disease: Pathogenic and therapeutic insights from toxin and genetic models” (Edited by Richard Nass and Serge Przeborski). Elsevier: San Diego.


Spampanato, J., Gu, X., Yang, X.W.*, Mody, I.* (2008). Progressive synaptic pathology of motor cortical neurons in a BAC transgenic mouse model of Huntington’s disease. Neuroscience 157, 606-620.  (*co-corresponding authors)


Gray, M., Shirasaki , D., Cepeda, C.,  Andre, V.M., Wilburn, B., Lu, X.H., Tao, J., Yamazaki, Y., Li, S.H., Sun, Y.E., Li, X.J., Levine, M.S., Yang, X.W. (2008). Full length human mutant Huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. J. Neuroscience 28, 6182-6195.


Lobo, M.K., Yeh, C., Yang, X.W. (2008). Pivotal role of early B-cell factor 1 in development of striatonigral medium spiny neurons in the matrix compartment. J Neurosci Res. 86, 2134-2146.



Lobo, M.K., Cui, Y., Ostlund, S.B., Balleine, B.W.*, Yang, X.W.* (2007).  Genetic control of instrumental conditioning by striatopallidal neuron-specific S1P receptor Gpr6. Nature Neuroscience 10, 1395-1397. (*co-corresponding authors)


Gu, X., Andre, V.M., Cepeda, C., Li, S.H., Li, X.J., Levine, M.S., Yang, X.W. (2007). Pathological cell-cell interactions are necessary for striatal pathogenesis in a conditional mouse model of Huntington’s disease. Molecular Neurodegeneration 2, 8.



Lobo, M.K., Karsten, S.L, Gray, M., Geschwind, D.H., Yang, X.W. (2006). FACS-array profiling of striatal projection neuron subtypes in juvenile and adult mouse brains. Nature Neuroscience 9, 443-452.

[Highlight as a Technical Breakthrough in Neuroscience in Nature]


Yang, Z., Jiang, H., Chachainasakul, T., Gong, S., Yang, X.W., Heintz, N., Lin, S. (2006). Modified Bacterial Artificial Chromosomes for zebrafish transgenesis. Methods 39, 183-188.



Gu, X., Li, C., Wei, W., Lo, V., Gong, S., Li, S., Iwasato, T., Itohara, S., Li, X., Mody, I., Heintz, N., Yang, X.W. (2005). Pathological cell-cell interactions elicited by a neuropathogenic form of mutant Huntingtin critically contribute to cortical pathogenesis in vivo. Neuron 46:433-444.

[NINDS Press Release]   [UCLA Press Releaese]


Yang, X.W., Gong, S. (2005). An overview on generation of BAC transgenic mice for neuroscience research. Current Protocols in Neuroscience, Unit 5.20.


Gong, S., Yang, X.W. (2005). Modification of Bacterial Artificial Chromosomes (BACs) and preparation of intact BAC DNA for generation of transgenic mice. Current Protocols in Neuroscience, Unit 5.21.


Before 2005:

Gong, S., Yang, X.W., Li, C., Heintz, N. (2002). Highly efficient modifications of Bacterial Artificial Chromosomes (BACs) using novel shuttle vectors containing the R6Kg origin of replication. Genome Research 12, 1992-1998.


Misulovin, Z., Yang, X.W., Yu, W., Heintz, N., Merfre, E. (2001). A rapid method for targeted modification and screening of recombinant Bacterial Artificial Chromosome. J. Immuno. Methods 257, 99-105.


Yang, X.W., Wynder, C., Doughty, M.L., Heintz, N. (1999). BAC mediated gene-dosage analysis reveals a role for Zipro1(Ru49/Zfp38) in progenitor cell proliferation in cerebellum and skin. Nature Genetics 22, 327-335.

[News and Views in Nature Genetics]


Yang, X.W., Model, P., Heintz, N. (1997). Homologous recombination based modification in E.Coli and germline transmission in transgenic mice of an 131kb Bacterial Artificial Chromosome (BAC). Nature Biotechnology 15, 859-865. [Cover Story]

[News and Views in Nature Biotechnology]


Yang, X.W., Zhong, R., Heintz, N. (1996). Granule cell specification in the developing mouse brain as defined by expression of the zinc finger transcription factor Ru49. Development 122: 555-566.


Baserga, S.J., Gilmore-Hebert, M., Yang, X.W. (1992). Distinct molecular signals for nuclear import of the nucleolar snRNA, U3. Genes & Development 6, 1120-1130.


Baserga, S.J., Yang, X.W., Steitz, J.A. (1991). An intact box C sequence in the U3 snRNA is required for binding of fibrillarin, the protein common to the major family of nucleolar snRNPs. EMBO J.10, 2645-2651.


Baserga, S.J., Yang, X.W., Steitz, J.A. (1991). Three pseudogenes for human U13 snRNA belong to class III. Gene 107, 347-348.


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