李晓江，中科院遗传与发育生物学研究所研究员，博士生导师。

E-mail： xjli@genetics.ac.cn

1982年，江西医学院医学专业学士；1986年，苏州医学院药理学硕士；1991年，美国Oregon Health Science University 药理学博士；1991-1995年，美国Johns Hopkins University 神经学科博士后；1995-1996年，美国Johns Hopkins University精神病学系助理教授；1996-2001年， 美国Emory大学人类遗传学系助理教授; 2001年, 美国Emory大学人类遗传学系终身副教授; 2005年起, 美国Emory大学人类遗传学系终身教授; 2007年起，美国Emory大学Distinguished Professor of Human Genetics (杰出讲席教授)； 2008年起任教育部长江讲座教授 （华中科技大学）；2010年入选中组部"千人计划"（中科院遗传发育所）。 李晓江博士还兼任华中科技大学，中南大学与苏州大学的客座教授。

主要研究方向有：1．遗传性神经退行性疾病的病理机制研究。2．细胞内转运与早期神经系统的发育。

实验室主页：http://surface.iphy.ac.cn/xjli/index.htm

近年的代表性研究论文:

Bhat KP, Yan S, Wang CE, Li SH, Li X-J. Differential ubiquitination and degradation of huntingtin fragments modulated by E3 ligase Ube3a. Proc Natl Acad Sci U S A, in press, 2014

Yang HQ, Wang GH, Sun HT, Shu RZ, Liu T, Wang CE, Liu ZM, Zhao Y, Zhen QY, Yang DS, Huang J, Zhou YL, Li SH, Jiang XD, Xiao ZC, Li X-J*, Lai LX. Species-dependent neuropathology in transgenic SOD1 pigs. Cell Res 2014 Feb 28. doi: 10.1038/cr.2014.25. [Epub ahead of print] (*Corresponding author)

Yan S, Wang CE, Wei W, Gaertig MA, Lai L, Li S, Li X-J. TDP-43 causes differential pathology in neuronal versus glial cells in the mouse brain. Hum Mol Genet. 2014 Jan 10. [Epub ahead of print]

Su Y, Huang SS, Gaertig MA, Li X-J*, Li SH. Age-dependent decrease in chaperone activity impairs MANF expression leading to Purkinje cell degeneration in inducible SCA17 mice. Neuron, 81, 349–365 2014 (*Corresponding author)

Xiang JX, Yang H, Zhao T, Sun M, Xu XS, Zhou X.F., Li SH, Li X-J. Huntingtin-Associated Protein 1 is essential for postnatal growth by regulating neurogenesis. J Clin Invest. 124(1):85-98. 2014

Xu Q.Q., Huang S.S., Song M.K., Wang C.E. Yan S., Liu X.D., Gaertig M.A., Yu. S.P., Li H., Li S.H., Li X-J. Synaptic mutant huntingtin inhibits synapsin-1 phosphorylation and causes neurological symptoms. J. Cell Biol. 202:1123-1138, 2013.

Weng L, Lin YF, Li AL, Wang CE, Yan S, Sun M, Gaertig MA, Mitha N, Kosaka J, Wakabayashi T, Xu X, Tang B, Li S, Li XJ. Loss of Ahi1 affects early development by impairing BM88/Cend1-mediated neuronal differentiation. J Neurosci. 33:8172-84. 2013.

Cape A, Chen XX, Wang CE, O'Neill A, Lin YF, He J, Xu XS, Yi H, Li H, Li SH, Li X-J. Loss of huntingtin-associated protein 1 impairs insulin secretion from pancreatic beta cells. Cell Mol Life Sci, 69:1305-17. 2012.

Wang H, Huang Z, Huang L, Niu S, Rao X, Xu J, Kong H, Yang J, Yang C, Wu D, Li S, Li XJ, Liu T, Sheng G. Hypothalamic Ahi1 mediates feeding behavior through interaction with 5-HT2C receptor. J Biol Chem. 287(3):2237-46. 2012

Mandal M, Wei J, Zhong P, Chen J, Duffney LJ, Liu W, Yuen EY, Twelvetrees AE, Li S, Li XJ, Kittler JT, Yan Z. Impaired AMPA receptor trafficking and function by mutant Huntingtin. J Biol Chem. 286:33719-28. 2011.

Huang SS, Ling JJ, Yang S. Li X-J*, Li SH. Neuronal expression of TATA box binding protein containing expanded polylgutamine in knock-in mice reduces chaperone protein response by impairing the function of NF-Y transcription factor. Brain 134(Pt 7):1943-58, 2011 (*Corresponding author)

Havel LS, Wang CE, Huang B, Wade B, Li SH, Li X-J. Preferential localization of N-terminal mutant huntingtin in striatal neurons cause neurological symptoms and is regulated by phosphorylation. Hum Mol Genet. 20:1424-37, 2011

Xu XS, Yang H, Lin Y.F., Li X, Cape A, Ressler KJ, Li SH, Li X-J. Neuronal Abelson helper integration site-1 (Ahi1) deficiency in mice alters TrkB signaling with a depressive phenotype.Proc Natl Acad Sci U S A. 107:19126-31, 2010

Yang DS, Wang CE, Zhao BT, Li W, Quyan Z, Liu ZM, Yang HQ, O'Neill A, Yi H, Li SH, Lai LX, Li X-J. Expression of Huntington disease protein results in apoptotic neurons in the brains of cloned transgenic pigs. Hum. Mol. Genet, 19:3983-94, 2010

Lin YF, Xu XS, Cape A, Li SH, Li XJ. Hap1 deficiency in orexin-producing neurons impairs neuronal process extension and leads to abnormal behavior in mice. J Biol Chem. 285:15941-9, 2010.

Bradford J, Shin J-Y, Roberts M, Wang C-E, Sheng G-Q, Li SH, Li X-J. Mutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease mice. J Biol Chem. 285:10653-61, 2010.

Bradford J, Shin J-Y, Roberts M, Wang C-E, Li X-J, Li SH. Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms. Proc Natl Acad Sci USA. 106:22480-5, 2009

Friedman MJ, Li S, Li X-J. Activation of gene transcription by heat shock protein 27 may contribute to its neuronal protection. J Biol Chem. 284:27944-51, 2009

Orr AG, Orr AL, Li X-J, Gross RE, Traynelis SF. Adenosine A2A receptor mediates microglial process retraction. Nature Neurosci. 12:872-8, 2009.

Shah A, Freidman M. Huang SS, Roberts M, Li X-J, Li SH Transcriptional dysregulation of TrkA associates with neurodegeneration in spinocerebellar ataxia type 17. Hum Mol Genet. 18: 4141-52, 2009.

Tydlacka S, Wang CE, Wang XJ, Li SH, Li X-J. Differential activities of the ubiquitin-proteasome system in neurons versus glia may account for the preferential accumulation of misfolded proteins in neurons. J. Neurosci. 28: 13285-13295, 2008

Wang CE, Tydlacka S, Adam OL, Yang SH, Graham RK, Hayden MR, Li SH, Chan AW, Li X-J. Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. Hum. Mol. Genet. 17: 2738-2751, 2008

Sheng GQ, Xu XS, Lin YF, Wang CE, Rong J, Peng J, Jiang X, Li SH, Li X-J. Huntingtin-associated protein 1 interacts with Ahi1 to regulate cerebellar and brainstem development in mice. J Clin Invest 118:2785-95, 2008

Wang CE, Zhou H, McGuire JR, Cerullo V, Lee B, Li SH, Li X-J. Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin. J. Cell Biol. 181: 803-816, 2008

Orr AL, Huang SS, Robert MA, Reed JC, Li SH, Li X-J. Sex-dependent effect of BAG1 in ameliorating motor deficits of huntington's disease transgenic mice. J. Biol. Chem. 283:16027-36, 2008

Yang SH, Cheng PH, Banta H, Piotrowska-Nitsche K, Yang JJ, Larkin K, Snyder B, Cheng ECH, Liu J, Orkin J, Fang ZH, Smith Y, Bachevalier J, Zola SM, Li SH, Li X-J, Chang AWS. Toward a transgenic model of Huntington's disease in a non-human primate. Nature 453: 921-924, 2008

Orr AL, Li SH, Wang CE, Li H, Rong J, Xu XS, Mastroberardino PJ, Greenamyre TJ, Li X-J. N-terminal Mutant Huntingtin Associates with Mitochondria Directly and Disrupts the Interaction of Mitochondria with Trafficking Proteins. J. Neurosci. 28: 2783-92, 2008

Wang JJ, Wang CE, Orr A, Tydlacka S, Li SH, Li X-J. Impaired ubiquitin-proteasome system activity in the synapses of Huntington disease mice. J. Cell Biol. 180: 1177-89, 2008.

Friedman MJ, Wang CE, Li XJ, Li SH. Polyglutamine expansion reduces the association of TBP with DNA and induces DNA binding-independent neurotoxicity. J Biol Chem. 283: 8283-90, 2008

Metzger S, Rong J, Nguyen HP, Cape A, Tomiuk J, Soehn A, Propping P, Freudenberg-Hua Y, Freudenberg J, Tong L, Li SH, Li XJ*, Riess O. Huntingtin-associated protein-1 is a modifier of the age-at-onset of Huntington's disease. Hum Mol Genet. 17: 1137-46, 2008 (*Corresponding author)

Friedman M., Fang ZH., Shah A., Goursac L., Warren S., Li SH., Li X-J. Polyglutamine domain regulates the TBP-TFIIB interaction: implications for its normal function and neurodegeneration. Nature Neuroscience 10: 1519-28, 2007