个人简历

李晓江教授，美国Oregon Health Sciences University 博士及 Johns Hopkins University博士后。自1996年以来，在美国Emory大学人类遗传学系任职并于2005晋升为终身正教授，2007年起为美国Emory大学人类遗传学系Distinguished Professor（杰出讲席教授）2011-2016年在中国科学院遗传与发育生物学研究所利用 CRISPR/Cas9等基因修饰技术制备重大疾病非人灵长类模型。2017年初加盟暨南大学，现任暨南大学粤港澳中枢神经再生研究院教授。
    李晓江教授是国际著名神经退行性疾病研究专家，于2008年与合作者在世界上首次利用转基因方法建立了非人灵长类的亨廷顿疾病模型。2015年制备出首例帕金森病的转基因猴模型，并利用CRISPR/Cas9技术制备了首例杜氏肌营养不良症及自闭症猴模型。2018年建立的世界首例亨廷顿基因敲入猪模型的研究成果发表在国际顶级期刊《Cell》杂志，受到了国内外的高度好评和关注。这些疾病包括亨廷顿疾病、老年痴呆疾病、帕金森疾病、肌萎缩侧索硬化症ALS疾病、自闭症等。研究成果发表于Cell, Nature, Nature Genetics, Nature Medicine, Nature Neuroscience, Neuron, J Clin Invest, Cell Stem Cell, Cell Research, PNAS, J Cell Biol, J Neurosci等国际核心刊物180篇，研究论文总影响因子（IF）达 1388， H index 为 63，累计引用率达到14221 余次。

研究领域

研究方向为重大脑疾病大动物（猴，猪）模型的建立及病理机制研究""

近期论文

2020
Yang S, Yang H, Huang L, Chen L, Qin Z, Li S, Li X-J. Lack of RAN-mediated toxicity in Huntington's disease knock-in mice. Proc Natl Acad Sci U S A. 2020 Feb 25;117(8):4411-4417.
Liu Q, Huang S, Yin P, Yang S, Zhang J, Jing L, Cheng S, Tang B, Li X-J, Pan Y, Li S. Cerebellum-enriched protein INPP5A contributes to selective neuropathology in mouse model of spinocerebellar ataxias type 17. Nat Commun. 2020 Feb 27;11(1):1101. doi: 10.1038/
Yin P, Liu Q, Pan Y, Yang W, Yang S, Wei W, Chen X, Hong Y, Bai D, Li X-J, Li S. Phosphorylation of myelin regulatory factor by PRKG2 mediates demyelination in Huntington's disease. EMBO Rep. 2020 Apr 9:e49783. doi: 10.15252.
Yang HM, Yang S, Jing L, Huang LX, Chen LX, Zhao XX, Yang WL, Pan YC, Yin P, Qin Z, Li SH, Li X-J. Truncation of mutant huntingtin in knock-in mice demonstrates exon1 huntingtin is a key pathogenic form. Nat Commun. 2020 May 22;11(1):2582.
Chen X, Xin N, Pan Y, Zhu L, Yin P, Liu Q, Yang W, Xu X, Li S, Li XJ. Huntingtin-Associated Protein 1 in Mouse Hypothalamus Stabilizes Glucocorticoid Receptor in Stress Response. Front Cell Neurosci. 2020 Jun 4;14:125. doi: 10.3389/fncel.2020.00125.
Liu Q, Cheng S, Yang H, Zhu L, Pan Y, Jing L, Tang B, Li S, Li XJ. Loss of Hap1 selectively promotes striatal degeneration in Huntington disease mice. Proc Natl Acad Sci U S A. 2020 Aug 3:202002283. doi: 10.1073/pnas.2002283117.
2019

Yang W, Liu YB, Tu ZC, Xiao C, Yan S, Ma XS, Guo XY, Chen XS, Yin P, Yang ZY, Yang S, Jiang TZ, Li SH, Qin C, Li X-J.CRISPR/Cas9-mediated PINK1 deletion leads to neurodegeneration in rhesus monkeys. Cell Res, 29(4):334-336 2019
Yin P, Gao XY, Yang WL, Yan S, Yang S, Zhao T, Q Sun, Liu YB, Li SH, Li X-J. Caspase-4 mediates cytoplasmic accumulation of mutant TDP-43 in the non-human primate brain. Acta Neuropathol 2019 137(6):919-937.
Yang W, Li SH, Li X-J. A CRISPR monkey model reveals a unique function of PINK1 in primate brains. Mol Neurodegener. 14:17, 2019
Zhu LY, Chen LQ, Yan LY Perkins BD, Li SH, Li BM, Xu HA, Li X-J. Mutant Ahi1 affects retinal axon projection in zebrafish via toxic gain of function. Front Cell Neurosci. 2019 Mar 21;13:81. doi: 10.3389/fncel.2019.00081. 2019
2018
Guo JF, Cui YT, Yang Y, Tang BS, Li YJ, Jin P, Li X-J, Yang S, Li SH. Piperine ameliorates SCA17 neuropathology via enhancing MANF expression and reducing ER stress. Mol Neurodegener. 2018 Jan 30;13(1):4.,
Yan S, Tu ZC, Liu ZM, Fan NN, Yang WL, Zhao Y, Ouyan Z, Lai CD, Yang HQ, Li L, Shi H, Xu GQ, Zhao H, Wei HJ, Pei Z, Li SH, Lai LX, Li X-J. A huntingtin knock-in pig model recapitulates features of selective neurodegeneration in Huntington’s disease. Cell173(4):989-1002. 2018
Tu ZC, Zhao H, Li B, Yan S, Wang L, Tang YJ, Li ZJ, Bai DZ, Li CJ, Lin YQ, Li YF, Liu JR, Xu H, Guo XY, Jiang YH, Zhang YQ, Li X-J. CRISPR/Cas9-mediated disruption of SHANK3 in monkey leads to drug-treatable autism-like symptoms. Hum Mol Genet, 2018; 28(4):561-571.
Yang S, Li S, Li X-J. Shortening the half-life of Cas9 maintains its gene editing ability and reduces neuronal toxicity. Cell Rep, 25, 2653–2659, 2018
2017
Tu ZC, Yang WL, Yan S, Yin A, Gao JQ, Liu XD, Zheng YH, Zheng JZ, Li ZJ, Yang S, Li SH, Guo XY, Li X-J. Promoting Cas9 degradation reduces mosaic mutations in non-human primate embryos. Sci Rep. 2017 Feb 3;7:42081. 2017
Zhao H, Tu ZC, Xu HJ, Yan S, Yan HH, Zheng YH, Yang WL, Zheng JH, Li ZJ, Tian R, Lu YM Gao XY, Jiang YH*, Li X-J*. Zhang YQ*. Altered neurogenesis and disrupted expression of synaptic proteins in prefrontal cortex of SHANK3-deficient non-human primate. Cell Res, 2017 Oct;27(10):1293-1297. (*corresponding authors)
Yang Y, Yang S, Guo JF, Cui YT, Tang BS, Li X-J, Li SH. Synergistic toxicity of polyglutamine-expanded TBP in glia and neuronal cells: therapeutic implications for SCA17. J Neurosci, 2017 Sep 20;37(38):9101-9115.
Li X-J, Tu ZC, Yang WL, Li SH. CRISPR: established editor of human embryos? Cell Stem Cell. 2017 Sep 7;21(3):295-296. 2017
Zhao T, Hong Y, Yin P, Li SH, Li-XJ. Differential HspBP1 expression accounts for the greater vulnerability of neurons than astrocytes to misfolded proteins. Proc. Natl. Acad . Sci. USA 2017 Sep 12;114(37):E7803-E7811.
Yang S, Yang HM Chang RB Yang Y, Yin P, Yang WL, Huang SS Geartig, MA, Li SH, Li X-J. MANF regulates hypothalamic control of food intake and body weight. Nature Commun, Sep 18;8(1):579. 2017.
Yang S, Chang RB, Yang HM, Zhao T, Hong Y, Kong HE Sun XB Qin ZH, Jin P, Li SH, Li X-J. CRISP R/Cas9-mediated gene editing ameliorates neurotoxicity in mouse model of Huntington’s disease. J Clin Invest. 127(7):2719-2724. 2017
Xiang JX, Yang S Xin N, Gaertig MA, Reeves RH Li SH, Li X-J. DYRK1A regulates Hap1-Dcaf7/WDR68 binding with implication for delayed growth in Down syndrome. Proc. Natl. Acad . Sci. USA 2017 Jan 30. pii: 201614893. doi: 10.1073/pnas.1614893114. [Epub ahead of print]. 2017