唐铁山，男，博士，现为膜生物学国家重点实验室研究员，“分子神经生物学研究组”研究组组长，中国科学院大学和中国科技大学兼职教授。1998年于中国科学院发育生物学研究所获得博士学位，随后留所任助理研究员。2000年以后在美国德克萨斯大学西南医学研究中心生理系做博士后研究和讲师。目前担任《神经药理学报》编委，中国老年学学会衰老与抗衰老科学委员会理事，北京分子生物学与生物化学学会和细胞生物学会理事。

　　主要研究方向为钙信号、线粒体以及DNA修复通路等缺陷与神经发育和神经退行性疾病的内在关系。在国际上首先发现了维持细胞钙稳态的保守离子通道和机制；阐明了钙信号紊乱参与亨廷顿氏舞蹈病和颅面畸形痴呆综合症病理发生的分子机制；发现稳定神经细胞的钙信号可以显著延缓舞蹈病的退行性病变；发现DNA修复通路缺陷与渐冻症ALS、脊髓小脑共济失调症SCAs等疾病的潜在联系。近年来所取得的研究成果发表在国际核心学术期刊，包括Cell、Neuron、Nucleic Acids Res、Prog Neurobiol、PNAS、J Cell Biol、Hum Mol Genetics、J Neuroscience、Epigenomics等。发表SCI收录论文近50篇，被引用3100余次，目前H-Index为28。本研究组将继续致力于以小鼠疾病模型为研究体系，利用分子、生化、细胞、遗传、组学等最新技术，研究钙信号紊乱、线粒体功能异常以及DNA损伤应答和修复缺陷与人类重大神经发育性/神经退行性疾病（如PD、CFT、ALS、SCAs）的关系；探索以钙信号通路、线粒体和DNA修复通路为切入点的神经疾病治疗方案和治疗药物。目前承担的项目主要有科技部重点专项、国家自然科学基金、中科院先导专项等。

　　目前指导在读硕士生1名，在读博士生4名，指导与中国科学技术大学联合培养研究生1名。已毕业博士生9名，硕士生6名（含3名与国科大和中国科大联合培养研究生）。

　　科技部国家重点研发计划、中国科学院先导项目、国家自然科学基金项目、北京市自然科学基金项目等。

1. 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  doi: 10.1016/j.molcel.2022.01.020. (in press)
2. 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)
3. 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)
4. 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. 
5. 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
6. 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.
7. 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)
8. 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.
9. 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.
10. 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.
11. 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.
12. 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.
13. 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.
14. 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.
15. 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.
16. 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.
17. 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.
18. 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)
19. 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.
20.  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.
21. 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.
22. 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.
23. 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)
24. Guo C, Tang TS, Friedberg EC. (2010) SnapShot: Nucleotide Excision Repair. Cell vol 140(5): 754-754.e1.
25. Wang H, Chen X, Li Y, Tang TS* and Bezprozvanny I* (2010) Tetrabenazine is neuroprotective in Huntington’s disease mice. Molecular Neurodegeneration, 5:18
26. 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.
27. 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
28. 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
29. 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.
30. 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.
31. 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.
32. 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.
33. 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.
34. 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.
35. Tang TS, Bezprozvanny I* (2004) Dopamine receptor-mediated Ca²⁺ signaling in striatal medium spiny neurons. J Biol Chem. 279 (40): 42082-42094.
36. 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.
37. 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.
38. 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.
39. 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.