Charlene Smith
Associate Project Scientist, Department of Psychiatry and Human Behavior, University of California, Irvine
Biography
Charlene Smith, Ph.D., is an Associate Project Scientist in the Department of Psychiatry and Human Behavior at the University of California, Irvine, where she works in the laboratory of Dr. Leslie Thompson. Smith's research focuses on Huntington's disease, investigating mitochondrial dysfunction and potential therapeutic approaches through CRISPR/Cas9 gene editing and antisense oligonucleotides in iPSC-derived neurons and organoids. Her work has been supported by multiple fellowships and grants from the Huntington's (formerly Hereditary) Disease Foundation, the Huntington's Disease Society of America, and the California Institute for Regenerative Medicine.
Smith has contributed to research examining the role of PIAS1, an E3 SUMO ligase, in Huntington's disease pathology, including its effects on transcription, DNA damage repair, mitochondrial function, and synaptic activity. Her work has involved collaborations with colleagues to perform cryo-electron tomography to characterize organelle phenotypes in patient-derived neurons and has explored developmental abnormalities in Huntington's disease iPSC-derived neuronal cultures. She has also contributed to projects investigating nuclear pore complex disruption and protein SUMOylation networks in Huntington's disease models. Smith served as Conference Chair for the Gordon Research Seminar on CAG triplet repeat disorders in 2019.
Smith received her Ph.D. from Cardiff University, where she was supported by an MRC Capacity Building Studentship in Stem Cell Science. Her doctoral research focused on direct programming of human induced pluripotent stem cell-derived neural stem cells into medium spiny neurons through transcription factor expression. She has taught neuroscience courses to undergraduates at UC Irvine and has been involved in science education outreach, including mentoring high school students and developing science programs for special education schools.
Return to topEducation
- PhD in Stem Cell Science, Cardiff University, 2014
- BSc (Hons) in Biomedical Sciences & Anatomy, Cardiff University, 2010
Distinctions
- Huntington's Disease Foundation Research Grant, 2024-2027
- HDSA Human Biology Fellowship, 2021-2023
- Hereditary Disease Foundation Postdoctoral Fellowship, 2017-2019, 2019-2021
- Gordon Research Seminar Chair, CAG Triplet Repeat Disorders, 2019
- California Institute for Regenerative Medicine (CIRM) Training Grant, 2015
- MRC Capacity Building Studentship in Stem Cell Science, 2010
Areas of Expertise
- Neurodegenerative Disease Mechanisms
- Induced Pluripotent Stem Cells
- Mitochondrial RNA Granules
- Mitochondria dysfunction
- Neuronal and organoid differentiation
Recent Publications
- Stocksdale JT, Leventhal MJ, Lam S, Xu YX, Wang YO, Wang KQ, Thomas R, Faghihmonzavi Z, Raghav Y, Smith C, Wu J, Miramontes R, Sarda K, Johnston H, Shin MG, Huang T, Foster M, Barch M, Amirani N, Paiz C, et al., “Intersecting impact of CAG repeat and huntingtin knockout in stem cell-derived cortical neurons” (opens in new tab), Neurobiology of Disease, vol. 210, pp. 106914, 2025.
- Kachemov M, Vaibhav V, Smith C, Sundararaman N, Heath M, Pendlebury DF, Matlock A, Lau A, Morozko E, Lim RG, Reidling J, Steffan JS, Van Eyk JE, Thompson LM, “Dysregulation of protein SUMOylation networks in Huntington's disease R6/2 mouse striatum” (opens in new tab), Brain, vol. 148, no. 4, pp. 1212-1227, 2025.
- Gu Y, Pope A, Smith C, Carmona C, Johnstone A, Shi L, Chen X, Santos S, Bacon-Brenes CC, Shoff T, Kleczko KM, Frydman J, Thompson LM, Mobley WC, Wu C, “BDNF and TRiC-inspired reagent rescue cortical synaptic deficits in a mouse model of Huntington's disease” (opens in new tab), Neurobiology of Disease, vol. 195, pp. 106502, 2024.
- Smith C., Steffan J.S., Thompson L.M., “SUMO modification in Huntington's disease: Unraveling complex mechanisms for therapeutic insights” (opens in new tab), Huntington S Disease Pathogenic Mechanisms and Implications for Therapeutics, pp. 259-283, 2024.
- Wu GH, Smith-Geater C, Galaz-Montoya JG, Gu Y, Gupte SR, Aviner R, Mitchell PG, Hsu J, Miramontes R, Wang KQ, Geller NR, Hou C, Danita C, Joubert LM, Schmid MF, Yeung S, Frydman J, Mobley W, Wu C, Thompson LM, et al., “CryoET reveals organelle phenotypes in huntington disease patient iPSC-derived and mouse primary neurons” (opens in new tab), Nat Commun, vol. 14, no. 1, pp. 692, 2023.
- Morelli KH, Wu Q, Gosztyla ML, Liu H, Yao M, Zhang C, Chen J, Marina RJ, Lee K, Jones KL, Huang MY, Li A, Smith-Geater C, Thompson LM, Duan W, Yeo GW, “An RNA-targeting CRISPR-Cas13d system alleviates disease-related phenotypes in Huntington's disease models” (opens in new tab), Nat Neurosci, vol. 26, no. 1, pp. 27-38, 2023.
- Olivarria GM, Cheng Y, Furman S, Pachow C, Hohsfield LA, Smith-Geater C, Miramontes R, Wu J, Burns MS, Tsourmas KI, Stocksdale J, Manlapaz C, Yong WH, Teijaro J, Edwards R, Green KN, Thompson LM, Lane TE, “Microglia Do Not Restrict SARS-CoV-2 Replication following Infection of the Central Nervous System of K18-Human ACE2 Transgenic Mice” (opens in new tab), J Virol, vol. 96, no. 4, pp. e0196921, 2022.
- Morozko EL, Smith-Geater C, Monteys AM, Pradhan S, Lim RG, Langfelder P, Kachemov M, Kulkarni JA, Zaifman J, Hill A, Stocksdale JT, Cullis PR, Wu J, Ochaba J, Miramontes R, Chakraborty A, Hazra TK, Lau A, St-Cyr S, Orellana I, et al., “PIAS1 modulates striatal transcription, DNA damage repair, and SUMOylation with relevance to Huntington's disease” (opens in new tab), Proc Natl Acad Sci U S A, vol. 118, no. 4, 2021.
- Smith-Geater C, Hernandez SJ, Lim RG, Adam M, Wu J, Stocksdale JT, Wassie BT, Gold MP, Wang KQ, Miramontes R, Kopan L, Orellana I, Joy S, Kemp PJ, Allen ND, Fraenkel E, Thompson LM, “Aberrant Development Corrected in Adult-Onset Huntington's Disease iSPC-Derived Neuronal Cultures via WNT Signaling Modulation” (opens in new tab), Stem Cell Reports, vol. 14, no. 3, pp. 406-419, 2020.
- Gao R, Chakraborty A, Geater C, Pradhan S, Gordon KL, Snowden J, Yuan S, Dickey AS, Choudhary S, Ashizawa T, Ellerby LM, La Spada AR, Thompson LM, Hazra TK, Sarkar PS, “Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription” (opens in new tab), Elife, vol. 8, 2019.
- Geater C, Hernandez S, Thompson L, Mattis VB, “Cellular Models: HD Patient-Derived Pluripotent Stem Cells” (opens in new tab), Methods Mol Biol, vol. 1780, pp. 41-73, 2018.
- Grima JC, Daigle JG, Arbez N, Cunningham KC, Zhang K, Ochaba J, Geater C, Morozko E, Stocksdale J, Glatzer JC, Pham JT, Ahmed I, Peng Q, Wadhwa H, Pletnikova O, Troncoso JC, Duan W, Snyder SH, Ranum LPW, Thompson LM, et al., “Mutant Huntingtin Disrupts the Nuclear Pore Complex” (opens in new tab), Neuron, vol. 94, no. 1, pp. 93-107.e6, 2017.
- Kemp PJ, Rushton DJ, Yarova PL, Schnell C, Geater C, Hancock JM, Wieland A, Hughes A, Badder L, Cope E, Riccardi D, Randall AD, Brown JT, Allen ND, Telezhkin V, “Improving and accelerating the differentiation and functional maturation of human stem cell-derived neurons: role of extracellular calcium and GABA” (opens in new tab), J Physiol, vol. 594, no. 22, pp. 6583-6594, 2016.
- Telezhkin V, Schnell C, Yarova P, Yung S, Cope E, Hughes A, Thompson BA, Sanders P, Geater C, Hancock JM, Joy S, Badder L, Connor-Robson N, Comella A, Straccia M, Bombau G, Brown JT, Canals JM, Randall AD, Allen ND, et al., “Forced cell cycle exit and modulation of GABAA, CREB, and GSK3β signaling promote functional maturation of induced pluripotent stem cell-derived neurons” (opens in new tab), Am J Physiol Cell Physiol, vol. 310, no. 7, pp. C520-41, 2016.
- Geater C, “Direct programming of neural progenitors into medium spiny neurons by transcription factor transfection” (opens in new tab), 2014. Thesis submitted to Cardiff University.
Contact Information
Email: cgeater@uci.edu
Phone: (949) 824-9386
Address: 845 Health Sciences Rd. Rm. 4105, Irvine, CA 92697, Irvine, CA 92697-1705
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Last updated on 3/11/2026.