唐鐵山，男，博士，現為膜生物學國家重點實驗室研究員，“分子神經生物學研究組”研究組組長，中國科學院大學和中國科技大學兼職教授。1998年於中國科學院發育生物學研究所獲得博士學位，隨後留所任助理研究員。2000年以後在美國德克薩斯大學西南醫學研究中心生理係做博士後研究和講師。目前擔任《神經藥理學報》編委，中國老年學學會衰老與抗衰老科學委員會理事，北京分子生物學與生物化學學會和細胞生物學會理事。

　　主要研究方向為鈣信號、線粒體以及DNA修複通路等缺陷與神經發育和神經退行性疾病的內在關係。在國際上首先發現了維持細胞鈣穩態的保守離子通道和機製；闡明了鈣信號紊亂參與亨廷頓氏舞蹈病和顱麵畸形癡呆綜合症病理發生的分子機製；發現穩定神經細胞的鈣信號可以顯著延緩舞蹈病的退行性病變；發現DNA修複通路缺陷與漸凍症ALS、脊髓小腦共濟失調症SCAs等疾病的潛在聯係。近年來所取得的研究成果發表在國際核心學術期刊，包括Cell、Neuron、Nucleic Acids Res、Prog Neurobiol、PNAS、J Cell Biol、Hum Mol Genetics、J Neuroscience、Epigenomics等。發表SCI收錄論文近50篇，被引用3100餘次，目前H-Index為28。本研究組將繼續致力於以小鼠疾病模型為研究體係，利用分子、生化、細胞、遺傳、組學等最新技術，研究鈣信號紊亂、線粒體功能異常以及DNA損傷應答和修複缺陷與人類重大神經發育性/神經退行性疾病（如PD、CFT、ALS、SCAs）的關係；探索以鈣信號通路、線粒體和DNA修複通路為切入點的神經疾病治療方案和治療藥物。目前承擔的項目主要有科技部重點專項、國家自然科學基金、中科院先導專項等。

　　目前指導在讀碩士生1名，在讀博士生4名，指導與中國科學技術大學聯合培養研究生1名。已畢業博士生9名，碩士生6名（含3名與國科大和中國科大聯合培養研究生）。

　　科技部國家重點研發計劃、中國科學院先導項目、國家自然科學基金項目、北京市自然科學基金項目等。

1. Fu H, Huang M, Wu H, Zheng H, Gong Y, Xing L, Gong J, An R, Li Q, Jie X, Ma X, Tang TS*, Guo C (2025) SART3 promotes homologous recombination repair by stimulating DNA-RNA hybrids removal and DNA end resection. Nat Commun 2025 Mar 06;16:2244. Doi: 10.1038/s41467-025-57599-8.
2. Chen JM, Shi G, Yu LL, Shan W, Sun JY, Guo AC, Wu JP, Tang TS*, Zhang XJ*, Wang Q*. (2025) 3-HKA Promotes Vascular Remodeling After Stroke by Modulating the Activation of A1/A2 Reactive Astrocytes. Adv Sci 2025 Jan 24:e2412667. doi: 10.1002/advs.202412667. Online ahead of print.
3. Jiang D#, Liu H#*, Li T#, Zhao S, Yang K, Yao F, Zhou B, Feng H, Wang S, Shen J, Tang J, Zhang YX, Wang Y, Guo C*, Tang TS* (2024) Agomirs upregulating carboxypeptidase E expression rescue hippocampal neurogenesis and memory deficits in Alzheimer's disease.  Transl Neurodegener. 2024 Apr 26;13(1):24. doi: 10.1186/s40035-024-00414-z.
4. Cong H#, Wang J#, Du N#, Song L, Wang R, Yang Y, Lei R, Tang TS*, Liu CM*, Zhu S* Han X* (2024) ITGB4/CD104 Mediates Zika Virus Attachment and Infection. Nat Commun. 2024 Dec 30;15:10729. Doi: 10.1038/s41467-024-54479-5
5. Wu W#, Fan Z#, Fu H#, Ma X#, Wang D, Liu H, Zhang C, Zheng H, Yang Y, Wu H, Miao X, An R, Gong Y, Tang TS*, Guo C* (2024) VGLL3 modulates chemosensitivity through promoting DNA double-strand break repair. Sci Adv. 2024 Oct 11;10(41):eadr2643. doi: 10.1126/sciadv.adr2643. Epub 2024 Oct 9.
6. Zhao S^#, Feng H^#, Jiang D^#, Yang K, Wang ST, Zhang YX, Wang Y, Liu H*, Guo C*, Tang TS* (2023) ER Ca²⁺ overload activates the IRE1α signaling and promotes cell survival. Cell Biosci. 2023 Jul 3;13(1):123. doi: 10.1186/s13578-023-01062-y.
7. Jiang D^#, Li T^#, Guo C, Tang TS*and Liu H* (2023) Small molecule modulators of chromatin remodeling: from neurodevelopment to neurodegeneration. Cell Biosci. 2023 Jan 16;13(1):10. doi: 10.1186/s13578-023-00953-4.  
8. Zhang C, Zhou B*, Gu F, Liu H, Wu H, Yao F, Zheng H, Fu H, Chong W, Cai S, Huang M, Ma X, Guo Z, Li T, Deng W, Zheng M, Ji Q, Zhao Y, Ma Y, Wang QE, Tang TS* and Guo C* (2022) Micropeptide PACMP Inhibition Elicits Synthetic-lethal Effects by Decreasing CtIP and Poly(ADP-ribosyl)ation. Mol Cell 2022 Apr 7;82(7):1297-1312.e8. doi: 10.1016/j.molcel.2022.01.020. Epub 2022 Feb 25.
9. Zheng S, Zhao D, Hou G, Zhao S, Zhang W, Wang X, Li L, Liang L, Tang TS, and Hu Y* (2022) iASPP suppresses Gp78-mediated TMCO1 degradation to maintain Ca²⁺ homeostasis and control tumor growth and drug resistance. Proc Natl Acad Sci USA. doi: 10.1073/pnas.2111380119. (in press)
10. Tu Y, Li X, Zhu X, Liu X, Guo C, Jia D*, Tang TS* (2020) Determining the Fate of Neurons in SCA3: ATX3, a Rising Decision Maker in Response to DNA Stresses and Beyond. Front Cell Dev Biol 2020 Dec 23;8:619911. doi: 10.3389/fcell.2020.619911. (invited review)
11. Wang JQ, Zhu S, Wang Y, Wang F, An C, Jiang D, Gao L, Tu Y, Zhu X, Wang Y, Liu H, Gong J, Sun Z, Wang X, Liu L, Yang K, Guo C*, Tang TS* (2019) Miro2 supplies a platform for Parkin translocation to damaged mitochondria. Science Bulletin, 64: 730–747. 
12. Huang M, Zhou B, Gong J, Xing L, Ma X, Wang F, Wu W, Shen H, Sun C, Zhu X, Yang Y, Sun Y, Liu Y, Tang TS*, Guo C* (2018) RNA-splicing factor SART3 regulates translesion DNA synthesis. Nucleic Acids Res 46(9): 4560-4574. doi: 10.1093/nar/gky220
13. Sun Z, Zhang H, Wang X, Wang QC, Zhang C, Wang JQ, Wang YH, An CQ, Yang KY, Wang Y, Gao F, Guo C*, Tang TS* (2018) TMCO1 is essential for ovarian follicle development by regulating ER Ca²⁺ store of granulosa cells. Cell Death Differ 25(9): 1686-1701. doi: 10.1038/s41418-018-0067-x.
14. Liu H, Tang TS, Guo C* (2018) Epigenetic profiles in polyglutamine disorders. Epigenomics 10(1): 9-25. doi: 10.2217/epi-2017-0089. (invited review)
15. Ma X, Liu H, Li J, Wang Y, Ding YH, Shen H, Yang Y, Sun C, Huang M, Tu Y, Liu Y, Zhao Y, Dong MQ, Xu P, Tang TS*, Guo C*. (2017) Polη O-GlcNAcylation governs genome integrity during translesion DNA synthesis.  Nat Commun 2017 Dec 5; 8(1): 1941. doi: 10.1038/s41467-017-02164-1.
16. Gong J, Huang M, Wang F, Ma X, Liu H, Tu Y, Xing L, Zhu X, Zheng H, Fang J, Li X, Wang Q, Wang J, Sun Z, Wang X, Wang Y, Guo C*, Tang TS* (2017) RBM45 competes with HDAC1 for binding to FUS in response to DNA damage. Nucleic Acids Res 45(22): 12862-12876. doi: 10.1093/nar/gkx1102.
17. Tu Y, Liu H, Zhu X, Shen H, Ma X, Wang F, Huang M, Gong J, Li X, Wang Y, Guo C*, Tang TS* (2017) Ataxin-3 promotes genome integrity by stabilizing Chk1. Nucleic Acids Res 45(8): 4532-4549. doi: 10.1093/nar/gkx095.
18. Liu H, Li X, Ning G, Zhu S, Ma X, Liu X, Liu C, Huang M, Schmitt I, Wüllner U, Niu Y, Guo C*, Wang Q*, Tang TS* (2016) The Machado–Joseph Disease Deubiquitinase Ataxin-3 Regulates the Stability and Apoptotic Function of p53. PLoS Biol 14(11): e2000733. doi:10.1371/journal.pbio.2000733.
19. Wang QC, Zheng Q, Tan H, Zhang B, Li X, Yang Y, Yu J, Liu Y, Chai H, Wang X, Sun Z, Wang JQ, Zhu S, Wang F, Yang M, Guo C, Wang H, Zheng Q, Li Y#, Chen Q#, Zhou A*, Tang TS* (2016) TMCO1 is an ER Ca²⁺ load-activated Ca²⁺ (CLAC) channel.  Cell 165(6): 1454-1466. doi: 10.1016/j.cell.2016.04.051.
20. Yang Y, Liu Z, Wang F, Temviriyanukul P, Ma X, Tu Y, Lv L, Lin YF, Huang M, Zhang T, Pei H, Chen BP, Jansen JG, de Wind N, Fischhaber PL, Friedberg EC, Tang TS*, Guo C* (2015) FANCD2 and REV1 cooperate in the protection of nascent DNA strands in response to replication stress. Nucleic Acids Res. 43(17):8325-8339. doi: 10.1093/nar/gkv737.
21. Li X, Liu H, Fischhaber PL*, Tang TS* (2015) Towards therapeutic targets for SCA3: insight into the role of Machado-Joseph disease protein ataxin-3 in misfolded proteins clearance. Progress in Neurobiology 132: 34-58. doi: 10.1016/j.pneurobio.2015.06.004.
22. Qin Y, Guo T, Li G, Tang TS, Zhao S, Jiao X, Gong J, Gao F, Guo C, Simpson JL, Chen ZJ* (2015) CSB-PGBD3 Mutations Cause Premature Ovarian Failure. PLoS Genet. 2015 Jul 28; 11(7):e1005419. doi: 10.1371/journal.pgen.1005419.
23. Xie W, Wang JQ, Wang QC, Wang Y, Yao S*, Tang TS* (2015) Adult neural progenitor cells from Huntington’s disease mouse brain exhibit increased proliferation and migration due to enhanced Ca²⁺ ROS signals. Cell Proliferation 48(5): 517-531.
24. Liu Y, Yang Y, Tang TS, Zhang H, Wang Z, Friedberg E, Yang W, Guo C*. (2014) Variants of mouse DNA polymerase κ reveal a mechanism of efficient and accurate translesion synthesis past a benzo[a]pyrene dG adduct. Proc Natl Acad Sci USA. 111(5):1789-1794. doi: 10.1073/pnas.1324168111.
25. Wang F, Fischhaber PL, Guo C, Tang TS* (2014) Epigenetic modifications as novel therapeutic targets for Huntington's disease. Epigenomics 6(3): 287-297. (invited review)
26. Lv L, Wang F, Ma X, Yang Y, Wang Z, Liu H, Li X, Liu Z, Zhang T, Huang M, Friedberg E, Tang TS, Guo C*. (2013) Mismatch repair protein MSH2 regulates translesion DNA synthesis following exposure of cells to UV radiation. Nucleic Acids Research 41(22): 10312-10322. doi: 10.1093/nar/gkt793.
27.  Wang F, Yang Y, Lin X, Wang JQ, Wu YS, Xie W, Wang D, Zhu S, Liao YQ, Sun Q, Yang YG, Luo HR, Guo C*, Han C*, Tang TS* (2013) Genome-wide loss of 5-hmC is a novel epigenetic feature of Huntington's disease. Human Molecular Genetics 22(18): 3641-3653.
28. Zhang X, Lv L, Chen Q, Yuan F, Zhang T, Yang Y, Zhang H, Wang Y, Qian L, Chen B, Zhang Y, Friedberg EC, Tang TS*, Guo C* (2013) Mouse DNA Polymerase Kappa Has A Functional Role in the Repair of DNA Strand Breaks. DNA Repair (Amst) 12(5):377-388.
29. Wang JQ, Chen Q, Wang X, Wang QC, Wang Y, Cheng HP, Guo C, Sun Q, Chen Q, Tang TS* (2013) Dysregulation of Mitochondrial Calcium Signaling and Superoxide Flashes Cause Mitochondrial Genomic DNA Damage in Huntington’s Disease. J Biol Chem 288(5): 3070-3084.
30. Wang Z, Wang F, Tang TS*, Guo C* (2012) The role of PARP1 in the DNA damage response and its application in tumor therapy. Front Med. 6(2):156-164. (invited review)
31. Guo C, Tang TS, Friedberg EC. (2010) SnapShot: Nucleotide Excision Repair. Cell vol 140(5): 754-754.e1.
32. Wang H, Chen X, Li Y, Tang TS* and Bezprozvanny I* (2010) Tetrabenazine is neuroprotective in Huntington’s disease mice. Molecular Neurodegeneration, 5:18
33. Tang TS, Guo C, Wang H, Chen X, Bezprozvanny I* (2009) Neuroprotective effects of inositol 1,4,5-trisphosphate receptor C-terminal fragment in a Huntington's disease mouse model. J Neuroscience 29(5):1257-1266.
34. Chen X, Tang TS, Tu H, Nelson O, Pook M, Hammer R, Nukina N, Bezprozvanny I* (2008) Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3. J Neuroscience vol 28:12713-12724
35. Schneider JW, Gao Z, Li S, Farooqi M, Tang TS, Bezprozvanny I, Frantz D, Hsieh J* (2008) Small molecule activation of neuronal cell fate. Nat Chem Biol Vol 4(7): 408-410
36. Guo C, Tang TS, Bienko M, Dikic I and Friedberg EC* (2008) Requirements for the interaction of mouse Polκ with ubiquitin, and its biological significance. J Biol Chem 283 (8): 4658-4664.
37. Tang TS, Chen X, Liu J, Bezprozvanny I* (2007) Dopaminergic signaling and apoptosis of medium spiny neurons in Huntington’s disease. J Neuroscience vol 27: 7899-7910.
38. Guo C, Sonoda E, Tang TS, Parker JL, Bielen AB, Takeda S, Ulrich HD, Friedberg EC. (2006) REV1 protein interacts with PCNA: significance of the REV1 BRCT domain in vitro and in vivo. Mol Cell 23(2):265-271.
39. Guo C, Tang TS, Bienko M, Sonoda E, Parker JL, Bielen AB, Takeda S, Ulrich HD, Dikic I, Friedberg EC. (2006) Ubiquitin-Binding Motifs in Rev1 Protein are Required for Its Role in the Tolerance of DNA Damage. Mol Cell Biol 26(23):8892-8900.
40. Tang TS, Slow E, Lupu V, Stavrovskaya IG, Sugimori M, Llinas R, Kristal BS, Hayden MR, Bezprozvanny I* (2005) Disturbed Ca²⁺ signaling and apoptosis of medium spiny neurons in Huntington's disease. Proc Natl Acad Sci USA. 102(7): 2602-2607.
41. Chen Y, Beffert U, Ertunc M, Tang TS, Kavalali ET, Bezprozvanny I, Herz J* (2005) Reelin modulates NMDA receptor activity in cortical neurons. J Neuroscience, Vol 25, pp 8209-8216.
42. Tang TS, Bezprozvanny I* (2004) Dopamine receptor-mediated Ca²⁺ signaling in striatal medium spiny neurons. J Biol Chem. 279 (40): 42082-42094.
43. Tu H, Tang TS, Wang Z, Bezprozvanny I* (2004) Association of type 1 inositol 1,4,5-trisphosphate receptor with AKAP9 (Yotiao) and protein kinase A. J Biol Chem. 279 (18): 9375-9382.
44. Tang TS, Tu H, Chan EY, Maximov A, Wang Z, Wellington CL, Hayden MR, Bezprozvanny I* (2003) Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) trisphosphate receptor type 1. Neuron 39(2): 227-239.
45. Tang, TS, Tu H, Wang Z, and Bezprozvanny I* (2003) Modulation of the type 1 inositol (1,4,5)-trisphosphate receptor function by PKA and PP1-alpha. J Neuroscience 23(2): 403-415.
46. Tang TS, Dong JB, Huang XY and Sun FZ (2000) Ca²⁺ oscillations induced by a cytosolic sperm protein factor are mediated by a maternal machinery which functions only once in mammalian eggs. Development 127(5): 1141-1150.