个人简介

李晓江，暨南大学粤港澳中枢神经再生研究院教授，广东省非人灵长类动物模型研究重点实验室主任，国家高层次引进人才，国家特聘专家。

李晓江于1991年获美国Oregon Health Sciences University 博士学位及 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。2008年获选教育部重点人才工程讲座教授。2010年入选国家高层次人才引进计划，2012-2016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大动物模型。2019年全职加盟暨南大学，现任暨南大学粤港澳中枢神经再生研究院教授。

李晓江教授致力于研究早期神经系统发育、衰老与神经退行性疾病，利用转基因疾病动物（鼠，猪，猴）模型揭示与神经系统发育的重要基因和基因变异如何引起与衰老有关的蛋白构像异常与神经细胞死亡。研究成果发表于《Cell》《Nature》等国际核心刊物200篇，研究论文累计引用率达到30210余次， H index 为 92。

近年来代表性论文

2023

Zhu LH, Li SH,  Li XJ, Yin P. Pathological insights from amyotrophic lateral sclerosis animal models: comparisons, limitations, and challenges. Transl Neurodegener.  In press 2023  

Tu Z, Yan S, Han BF, Li CJ, Liang WA, Lin YQ, Ding YY, Wei HY, Wang L, Xu H, Ye JM, Li B, Li SH, Li XJ. Tauopathy promotes spinal cord-dependent production of toxic amyloid-beta in transgenic monkeys. Signal Transduct Target Ther In press 2023  

Li CJ, Lin YQ, Chen YZ, Song XC, Zheng X, Li JW, He J, Chen XS, Huang CH, Wang W, Wu JH, Wu JX, Gao JL, Tu ZC, Li XJ, Yan S, Li SH. A Specific Mini-intrabody Mediates Lysosome Degradation of Mutant Huntingtin. Adv Sci. In press, 2023

Yan S, Zheng X, Lin YQ, Li CJ, Liu ZM, Li JW, Tu ZC, Zhao Y, Huang CH, Chen YZ, Li J, Song XC, Han BF, Wang W, Liang WE, Lai LX, Li XJ, LI SH. Cas9-mediated replacement of expanded CAG repeats in a pig model of Huntington’s disease. Nat Biomed Eng. 2023 Feb 16. doi: 10.1038/s41551-023-01007-3. Online ahead of print.

Li B, Zhao H, Tu Z, Yang W, Han R, Wang L, Luo X, Pan M, Chen X, Zhang J, Xu H, Guo X, Yan S, Yin P, Zhao Z, Liu J, Luo Y, Li Y, Yang Z, Zhang B, Tan Z, Xu H, Jiang T, Jiang YH, Li S, Zhang YQ, Li XJ. CHD8 mutations increase gliogenesis to enlarge brain size in the nonhuman primate. Cell Discov. 2023 Mar 7;9(1):27.

Bai D, Zhu L, Jia Q, Duan X, Chen L, Wang X, Hou J, Jiang G, Yang S, Li S, Li XJ, Yin P. Loss of TDP-43 promotes somatic CAG repeat expansion in Huntington's disease knock-in mice. Prog Neurobiol. 2023 Jun 12:102484.

2022

Yang W, Guo X, Tu Z, Chen X, Han R, Liu Y, Yan S, Wang Q, Wang Z, Zhao X, Zhang Y, Xiong X, Yang H, Yin P, Wan H, Chen X, Guo J, Yan XX, Liao L, Li S, Li XJ. PINK1 kinase dysfunction triggers neurodegeneration in the primate brain without impacting mitochondrial homeostasis. Protein and Cell   2022 Jan;13(1):26-46. Epub 2021 Nov 20

Chen XX, Su YZ, Chen LQ, Che XS, Pan MT, Zhang YR, Wang Q, Yang WL, Yin P, He DJ, Guo XY, Yang S, Zhen Y, Yan S, Li XJ, LI SH. Differential expression and roles of Huntingtin and Huntingtin-associated protein 1 in the mouse and primate brains. Cell Mol Life Sci. 2022 Oct 17;79(11):554.

Han R, Liu YT, Li SH, Li XJ, Yang WL. PINK1/Parkin-mediated mitophagy: differences between in vitro and in vivo models. Autophagy. 2022 Nov 3:1-10.

Yang WL, Li S, Li XJ. AHI1: linking depression and impaired antiviral immune response. Cell Res. 2022 Oct;32(10):869-870.

Yin P, Li S, Li XJ, Yang W. New pathogenic insights from large animal models of neurodegenerative diseases. Protein Cell. 2022 Oct;13(10):707-720.

2021

Yin P, Bai DZ, Deng FY, Zhang C, Jia QQ, Zhu LH, Chen LQ, Li B, Guo XY, Ye JM, Tan ZQ, Wang L, Li SH, Li X-J. SQSTM1 mediated clearance of cytoplasmic mutant TARDBP/TDP-43 in the monkey brain. Autophagy (IF 16.016 ) Dec 22:1-14. doi: 10.1080/15548627.2021.2013653. Online ahead of print. 2021

Yang WL, Chen XS, Li SH, Li XJ. Genetically modified large animal models for investigating neurodegenerative diseases. Cell & Bioscience (IF 6.07). Dec 21;11(1):218, 2022.

Yang W, Guo X, Tu Z, Chen X, Han R, Liu Y, Yan S, Wang Q, Wang Z, Zhao X, Zhang Y, Xiong X, Yang H, Yin P, Wan H, Chen X, Guo J, Yan XX, Liao L, Li S, Li XJ. PINK1 kinase dysfunction triggers neurodegeneration in the primate brain without impacting mitochondrial homeostasis. Protein and Cell (IF 14.87)  2021 Nov 20. doi: 10.1007/s13238-021-00888-x.

Yin P, Bai D, Zhu L, Deng F, Guo X, Li B, Chen L, Li S, Li XJ. Cytoplasmic TDP-43 impairs the activity of the ubiquitin-proteasome system. Exp Neurol (IF 5.33). 2021 Aug 4:113833.

Jing L, Cheng S, Pan Y, Liu Q, Yang W, Li S, Li XJ. Accumulation of endogenous mutant huntingtin in astrocytes exacerbates neuropathology of Huntington disease in mice. Mol Neurobiol (IF 5.590). 2021 Jul 12. doi: 10.1007/s12035-021-02451-5. Online ahead of print.

Bai D, Yin P, Zhang Y, Sun F, Chen L, Lin L, Yan S, Li S, Li XJ. Lack of association of somatic CAG repeat expansion with striatal neurodegeneration in HD knock-in animal models. Hum Mol Genet (IF 6.15). 2021 May 5:ddab129.

Wang B, Xin N, Qian X, Zhai L, Miao Z, Yang Y, Li S, Sun M, Xu X, Li XJ. Ahi1 regulates the nuclear translocation of glucocorticoid receptor to modulate stress response. Transl Psychiatry (IF 6.222). 2021 Mar 29;11(1):188.

Zhang XY, Li J, Li CJ, Lin YQ, Huang CH, Zheng X, Song XC, Tu ZC, Li XJ, Yan S. Differential development and electrophysiological activity in cultured cortical neurons from the mouse and cynomolgus monkey. Neural Regen Res. (IF 5.135) 2021 Dec;16(12):2446-2452.

2020

Liu Q, Cheng SY, Yang HM, Zhu LY, Pan YC, Jing L, Tang BS, Li SH, Li X-J. Loss of Hap1 selectively promotes striatal degeneration in Huntington disease mic. Proc Natl Acad Sci U S A. 2020, 117(33):20265-20273.

Chen XX, Xin N, Pan YC, Zhu LY, Yin P, Liu Q, Yang WL, Xu XS, Li Sh, Li XJ. Huntingtin-associated protein 1 in mouse hypothalamus stabilizes glucocorticoid receptor in stress response. Front Cell Neurosci. Jun 4;14:125, 2020

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.

Yin P, Liu Q, Pan Y, Yang W, Yang S, Wei W, Chen X, Hong Y, Bai D, Li XJ, Li S.  Phosphorylation of myelin regulatory factor by PRKG2 mediates demyelination in Huntington's disease.  EMBO Rep. 2020 Apr 9:e49783. doi: 10.15252.

Liu Q, Huang S, Yin P, Yang S, Zhang J, Jing L, Cheng S, Tang B, Li XJ, 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/

Yang S, Yang H, Huang L, Chen L, Qin Z, Li S, Li XJ. 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.

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

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, 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

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

2018

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

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.

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. Cell 173(4):989-1002. 2018

Guo JF, Cui YT, Yang Y, Tang BS, Li YJ, Jin P, Li XJ, Yang S, Li SH. Piperine ameliorates SCA17 neuropathology via enhancing MANF expression and reducing ER stress. Mol Neurodegener. 2018 Jan 30;13(1):4.,

2017

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.

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 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.

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