论文
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| • Zhai, R.*,Tong, G., Li, Z., Song, W., Hu, Y.,Xu, S., Wei, Q., Zhang, X., Li, Y., Liao, B., Yuan, C., Fan, Y., Song, G., Ouyang, Y., Zhang, W., Tang, Y., Jin, M., Zhang, Y., Li, H., Yang, Z., Lin, G., Stein, D., Xiong, Z.*, Wang, Z.* (2023) Rhesus monkeys exhibiting spontaneous ritualistic behaviors resembling obsessive-compulsive disorder Natl Sci Rev. : In Press. |
| • Zhu, Z., Li, Y., Xu, J., Xue, H., Feng, X., Zhu, Y.*, Xiong, Z.* (2023) CDKL5 deficiency in adult glutamatergic neurons alters synaptic activity and causes spontaneous seizures via TrkB signaling. Cell Rep. 42(10): 113202. |
| • Xu, R., Wu, X., Xiong, Z.* (2023) Low-intensity ultrasound directly modulates neural activity of the cerebellar cortex. Brain Stimul. 16: 918-926. |
| • Xu, X., Sun, H., Luo, J., Cheng, X., Lv, W., Luo, W., Chen, W., Xiong, Z., Liu, JY.* (2022) The Pathology of Primary Familial Brain Calcification: Implications for Treatment. Neurosci. Bull. : In Press.（Review） |
| • Zhao, M., Su, H., Zeng, Y., Sun, Y., Guo, X., Li, Y., Wang, C., Zhao, Z., Huang, X., Lin, K., Ye, Z., Lin, B., Hong, S., Zheng, J., Liu, Y., Yao, X., Yang, D., Lu, Y., Chen, H., Zuo, E., Yang, G., Wang, H., Huang, C., Lin, X., Cen, Z., Lai, L., Zhang, Y., Li, X., Lai, T., Lin, L., Zuo, D., Lin, M., Liou, C., Kong, Q., Yan, C., Xiong, Z., Wang, N., Luo, W., Zhao, C.*, Cheng, X.*, Chen, W.* (2022) Loss of function of CMPK2 causes mitochondria deficiency and brain calcification. Cell Discov. 8: 128. |
| • Lu, Z., He, S., Jiang, J., Zhuang, L., Wang, Y., Yang, G., Jiang, X., Nie, Y., Fu, J., Zhang, X., Lu, Y., Bian, X., Chang, H., Xiong, Z., Huang, X*.,Liu, Z*., Sun, Q*. (2022) Base-edited cynomolgus monkeys mimic core symptoms of STXBP1 encephalopathy. Mol. Ther. 30: 2163-2175. |
| • Lu, B., Lou, S., Xu, R., Kong, D., Wu, R., Zhang, J., Zhuang, L., Wu, X., He, J., Wu, Z.,Xiong, Z.* (2021) Cerebellar spreading depolarization mediates paroxysmal movement disorder. Cell Rep. 36: 109743 |
| • Liu, Z., Li, K., Cai, Y., Liu, Q., Zhang, T., Lu, Y., Nie, Y., Wang, Y., Yan, Z., Qu, Y., Li, Y., Wang, Z., Xiong, Z.* and Qiang, S. (2021) Accelerated passage of gene-modified monkeys by hormone-induced precocious puberty Natl. Sci. Rev. 8: nwab083 |
| • Wang, H., Zhu, Z., Li, Y., Lou, S., Yang, G., Feng, X., Xu, W., Huang, Z., Cheng, X., Xiong, Z.* (2021) CDKL5 deficiency in forebrain glutamatergic neurons results in recurrent spontaneous seizures. Epilepsia. 62: 517-528 |
| • Qin, X., Shan, Q., Fang, H., Wang, Y., Chen, P.,Xiong, Z.*, Swaab, D. and Zhou, J. (2021) PSD-93 up-regulates the synaptic activity of corticotropin-releasing hormone neurons in the paraventricular nucleus in depression. Acta. Neuropathol. In Press: |
| • Li, H., Chen, Y., Zhuang, L., Chen, D., Ke, H., Luo, W., Cheng, H., Zhang, Y., Liu, Z., Chen, W., Wang, N., Wu, S., Zhou, W., Xiong, Z.* and Wu, Z. (2021) TMEM151A variants cause paroxysmal kinesigenic dyskinesias. Cell Discovery. 17: 83 |
| • Wang, F., Wang, Q., Liu, B., Mei, L., Ma, S., Wang, S., Wang, R., Zhang, Y., Niu, C.,Xiong, Z.*, Zheng, Y., Zhang, Z., Shi, J., and Song, X. (2021) The long noncoding RNA Synage regulates synapse stability and neuronal function in the cerebellum. Cell Death and Differentiation In Press: |
| • Liu, X., Chen, X., Zhao, M., Wang, Z., Chen, H., Li, H., Wang, C., Wu, S. Peng, C., Yin, Y., Fu, H., Lin, M., Yu, L.,Xiong, Z.*, Wu, Z. and Wang, N. (2021) CHIP control degradation of mutant ETF: QO through ubiquitylation in late-onset multiple acyl-CoA dehydrogenase deficiency. J. Inherit. Metab. Dis. 44: 450-468 |
| • Chen WJ, Cheng XW, Fu Y, Zhao M, McGinley J, Westenberger A and Xiong ZQ (2020) Rethinking monogenic neurological diseases. BMJ. 371: m3752 |
| • Liu, J., Pan, L., Shang, C., Lu, B., Wu, R., Feng, Y., Chen, W., Zhang, R., Bu, J., Xiong, Z.*, Bu, W.*, Du, J.*, and Shi, J.* (2020) A highly sensitive and selective nanosensor for near-infrared potassium imaging. Sci. Adv. 6: eaax9757 |
| • Lin, X., Su, H., Dong, E., Lin, X., Zhao, M., Yang, C., Wang, C., Wang, J., Chen, Y., Yu, H., Xu, J., Ma, X., Xiong, Z.*, Wang, N. and Chen, W. (2019) Stop-gain mutations in UBAP1 cause pure autosomal dominant spastic paraplegia. Brain 142: 2238-2252 |
| • Wang, T., Wang, J., Wang, J., Mao, L., Tang, B., Vanderklish, P., Liao, X.,Xiong, Z.* and Liao, L. (2019) HAP1 is an in vivo UBE3A target that augments autophagy in a mouse model of Angelman syndrome. Neurobiol. Dis. 132: 104585 |
| • Zhu, Z., Liu, J., Yang, C., Zhao, M., Xiong, Z. (2019) XPO1 inhibitor KPT-330 synergizes with Bcl-xL inhibitor to induce cancer cell apoptosis by perturbing rRNA processing and Mcl-1 protein synthesis. Cell Death Dis. 10: 395 |
| • Zhu, Z., Liu, J., Yang, C., Li, M., Wu, R., Xiong, Z. (2019) Targeting KPNB1 overcomes TRAIL resistance by regulating DR5, Mcl-1 and FLIP in glioblastoma cells. Cell Death Dis. 10: 118 |
| • Wang, J,Lou, S.,Wang, T.,Wu, R.,Li, G.,Zhao, M,Lu, B,Li, Y.,Zhang, J,Cheng, X,Shen, Y,Wang, X, Zhu, Z.,Li, M.,Takumi, T,Yang, H,Yu, X,Liao, L,Xiong, Z. (2019) UBE3A-mediated PTPA ubiquitination and degradation regulate PP2A activity and dendritic spine morphology. Proc. Natl. Acad. Sci. USA. 18: 12500-12505 |
| • Zhu, Y. and Xiong, Z.* (2019) Molecular and synaptic bases of CDKL5 disorder. Dev. Neurobiol. 79: 8-14 |
| • Xu, Q., Liu, Y., Wang, X., Tan, G., Li, H. Hulbert, S., Li, C., Hu, CC,Xiong, Z.*, Xu X, and Jiang YH. (2018) Autism-associated CHD8 deficiency impairs axon development and migration of cortical neurons. Mol. Autism. 9: 65 |
| • Zhu, Z.,Liu, J.,Li, K.,Zheng, J.,Xiong, Z. (2018) KPNB1 inhibition disrupts proteostasis and triggers unfolded protein response-mediatedapoptosis in glioblastoma cells. Oncogene 37: 2936-2952 |
| • Tan, G., Liu, Y., Wang, L., Li, K., Zhang, Z., Li, H., Yang, F., Li, Y., Li, D., Wu, M., Yu, C., Long, J., Chen, R., Li, L., Yin, L., Liu, J., Cheng, X., Shen, Q., Shu, Y., Sakimura, K., Liao, L., Wu, Z. and Xiong, Z.* (2018) PRRT2 deficiency induces paroxysmal kinesigenic dyskinesia by regulating synaptic transmission in cerebellum. Cell Res. 28: 90-110 |
| • Xu, X., Li, C., Gao, X., Xia, K., Guo, H., Li, Y., Hao, Z., Zhang, L., Gao, D., Xu, C., Xu, H.,Xiong, Z.*, Qiu, Z., Mei, L., Xie, X., Ruan, K. and Hu, R*. (2018) Excessive UBE3A dosage impairs retinoic acid signaling and synaptic plasticity in autism spectrum disorders. Cell. Res. 28: 48-68 |
| • Yao, X., Cheng, X., Wang, C., Zhao, M., Guo, X., Su, H., Lai, L., Zou, X., Chen, X., Zhao, Y., Dong, E., Lu, Y., Wu, S., Li, X., Fan, G., Yu, H., Xu, J., Wang, N., Xiong. Z., Chen. W. (2018) Biallelic Mutations in MYORG Cause Autosomal Recessive Primary Familial Brain Calcification. Neuron 98: 1116-1123 |
| • Liu, J., Zhu, Z., Li, K., Wang, H.,Xiong, Z.*, and Zheng, J. (2017) UMI-77 primes glioma cells for TRAIL-induced apoptosis by unsequestering Bim and Bak from Mcl-1. Mol Cell Biochem. 432: 55-65 |
| • Zuo, E., Cai, Y., Li, K., Wei, Y., Wang, B., Sun, Y., Liu, Z., Liu, J., Hu, X., Wei, W., Huo, X., Shi, L., Tang, C., Liang, D., Wang, Y., Nie, Y., Zhang, C., Yao, X., Wang, X., Zhou, C., Ying, W., Wang, Q., Chen, R., Shen, Q., Xu, G., Li, J.,Sun, Q.*,Xiong, Z* and Yang, H.* (2017) One-step generation of complete gene knockout mice and monkeys by CRISPR/Cas9-mediated gene editing with multiple sgRNAs. Cell Res. 27: 933-945. |
| • Yang, Z., Li, P., Chen, R., Wang, J., Wang, S., Shen, Y., Wu, X., Fang, B., Cheng, X., Xiong, Z. (2017) ADAM10-initiated release of Notch intracellular domain regulates microtubule stability and radial migration of cortical neurons. Cerebral Cortex. 27: 919-932 |
| • Li, W., Yao, A., Zhi, H., Kaur, K., Zhu, Y., Jia, M., Zhao, H., Wang, Q., Jin, S., Zhao, G.,Xiong, Z. and Zhang, Y. (2016) Angelman Syndrome Protein Ube3a Regulates Synaptic Growth and Endocytosis by Inhibiting BMP Signaling in Drosophila. PLoS Genet. 12: e1006062 |
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| • Poo, M., Du, J., Ip, N.,Xiong, Z., Xu, B., Tan, T. (2016) China Brain Project: Basic Neuroscience, Brain Diseases, and Brain-Inspired Computing. Neuron. 92: 591-596 |
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| • Li, Y., Wang, J., Zhou, Y., Li, D. and Xiong, Z. (2015) Rcan1 deficiency impairs neuronal migration and causes periventricular heterotopia. J. Neurosci. 35: 610-620 |
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| • Lu, T., Chen, R., COX, T., Moldrich, R., Kurniawan, N., Tan, G., Perry, J., Ashworth, A., Bartlett, P., Xu, L., Zhang, J., Lu, B., Wu, M., Shen, Q., Liu, Y., Richards, L. and Xiong, Z. (2013) X-linked microtubule-associated protein, Mid1, regulates axon development. Proc. Natl. Acad. Sci. USA. 110: 19131-19136 |
| • Zhu, Y., Li, D., Wang, L., Lu, B., Zheng, J., Zhao, S., Zeng, R. and Xiong, Z. (2013) Palmitoylation-dependent CDKL5-PSD-95 interaction regulates synaptic targeting of CDKL5 and dendritic spine development. Proc. Natl. Acad. Sci. USA. 110: 9118-9123 |
| • Miao, S., Chen, R., Ye, J., Tan, G., Li, S., Zhang, J., Jiang, Y. and Xiong, Z. (2013) The Angelman Syndrome protein Ube3a is required for polarized dendrite morphogenesis in pyramidal neurons. J. Neurosci. 33: 327-333 |
| • Tan, G., Liu, Y., Hu, X., Yin, D., Mei, L. and Xiong, Z. (2012) Neuregulin 1 represses limbic epileptogenesis through ErbB4 in parvalbumin-expressing interneurons. Nature Neuroscience. 15: 258-266 |
| • Guo, S., Tan, G., Li, S.,Cheng, X., Zhou, Y., Jia, Y., Xiong, H., Tao, J. and Xiong, Z. (2012) Serum inducible kinase is a positive regulator of cortical dendrite development and is required for BDNF-promoted dendritic arborization. Cell Research. 22: 387-398 |
| • Hu, X., Chen, X., Fei, J. and Xiong, Z. (2011) Neuron-restrictive silencer factor is not required for the antiepileptic effect of the ketogenic diet. Epilepsia. 52: 1609-1616 |
| • Hu, X., Chen, X., Cai, L., Tan, G., Xu, L., Feng, X., Lu, T., Xiong, H., Fei, J. and Xiong, Z. (2011) Conditional deletion of NRSF in forebrain neurons accelerates epileptogenesis in the kindling model. Cerebral Cortex. 21: 2158-2165 |
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